Lichen Nitidus

Chronic eruption which typically presents as multiple, grouped, asymptomatic, pinpoint-sized, flesh-colored papules in children and young adult men Occurs most commonly on the upper extremities and genitalia Lymphohistiocytic infiltrate filling the dermal papilla and obscuring the dermoepidermal junction (Fig. 12) Claw-like hyperplasia of the rete surround the infiltrate (Fig. 12) Epithelioid and multinucleated histiocytes may be present, or the infiltrate may be frankly granulomatous (Fig....

Histopathology

Palisades of histiocytes and focal and or intersecting bands of granulomatous dermatitis around extensive necrobiosis in the mid and deep dermis with extension into and through the subcutaneous lobules (Figs. 14B and C) Numerous foam cells and giant cells (Touton and foreign body giant cells) (Fig. 14D) Extracellular deposits of lipids and cholesterol clefts Perivascular (superficial and deep dermal and subcutaneous) lymphoplasmacytic infiltrate with lymphoid follicles

Differential Diagnosis

The superficial aspects of a syringoma may be difficult to distinguish from the superficial aspects of an MAC, especially in a shave biopsy. Sometimes, a dermatopathologist will find it necessary to defer to a deeper biopsy for a definitive diagnosis to be rendered. When the interpreter of a superficial biopsy is strongly considering the diagnosis of syrin-goma in a lesion that shows cornification and involvement of the deep biopsy margin, the clue of the sesame seed bun should be considered....

Postinflammatory Pigmentary Alteration

Synonyms Postinflammatory pigmentary change. Pigmented macules and patches Sites of a previous dermatitis More prominent in dark-skinned individuals Fading gradually over months or years Little or no infiltrate of lymphocytes around venules of the superficial plexus and along the dermoepidermal junction Hints of vacuolar alteration sometimes Melanophages in the papillary dermis and in the upper part of the reticular dermis range from few to many Papillary dermis thickened sometimes by subtle...

Pseudoporphyria

Like porphyria, but characteristic features downgraded Less prominent PAS+ deposits around vessels Immunofluorescence DIF mantle of Ig deposits (all classes) around vessels and at basement membrane zone in both porphyria and pseudoporphyria, due to nonspecific sticking, not autoimmune basis (Fig. 12F). Granular C5b-9 in vessel walls IIF no circulating antibodies Porphyrias are photoactive molecules that absorb in the visible violet light spectrum, and mediate oxidative damage to molecules in...

Info

In those variants of tuberculosis of the skin seen in association with caseation necrosis (most commonly primary, miliary, or scrofuloderma) one can also clinically discern ulceration with drainage from the granulomatous site. Cutaneous tuberculosis can occur by three routes direct inoculation (primary), hematogenous spread (lupus vulgaris, miliary tuberculosis), or by direct extension of an underlying tuberculous lymph node or bone (scrofuloderma). Lupus vulgaris usually occurs in previously...

Clinical Presentation

Skin colored or pink to red papules classically arranged in an annular or arciform pattern (Fig. 10A) Most commonly located on the hands, arms, feet, and legs Hyperpigmentation or normal skin noted in the center of the annular or circular lesions A generalized form is composed of widely distributed papules symmetrically on the trunk and extremities Subcutaneous, perforating, and patch-plaque variants have been described

Ectopic Sebaceous Glands

Synonyms Nevus sebaceus of Jadassohn organoid nevus. Usually on the scalp or face In infancy and childhood well-circumscribed, only slightly raised, hairless plaque in an often linear configuration (Fig. 3A) At puberty and in adulthood yellow verrucous, nodular plaque (Fig. 4A) Acanthotic epidermis with papillomatosis and hyper-keratosis (Figs. 3C 4B and C) Lack of normal mature hair follicles (Figs. 3B and C 4B and C) Incompletely differentiated (immature) hair follicles (Figs. 3C and D)...

Pathophysiology

Sarcoid is a multisystem systemic granulomatous disease. Approximately 25 to 33 of the patients demonstrate cutaneous manifestations. The exact etiology of sarcoidosis remains unknown but there is a known association with increased cell-mediated immunity activity and an increase in CD4+ T-helper cells of the Th1 subtype after antigen presentation. Th1 cytokines are increased (including inter-leukin 2 and interferon), which ultimately leads to B-cell stimulation. Granuloma forming T lymphocytes...

References

Melanocytic acral nevi with intraepidermal ascent of cells (MANIACs) a reappraisal of melanocytic lesions from acral sites abstr . J Cutan Pathol 1991 18 378. 2. Boyd AS, Rapini RP. Acral melanocytic neoplasms a histologic analysis of 158 lesions. J Am Acad Dermatol 1994 31 740-745. 3. Fallowfield ME, Collina G, Cook MG. Melanocytic lesions of the palm and sole. Histopathol 1994 24 463-467. 4. Clemente C, Zurrida S, Bartoli C, Bono A, Collini P, Rilke F....

Lentigo Maligna Melanoma Clinical Presentation

LMM is an invasive melanoma that develops from a precursor lesion termed lentigo maligna. LM usually occurs on the sun-damaged skin of the head or neck, especially over bony prominences such as the forehead, bald scalp, or malar areas. The initial lesion is a faint tan macule. Over time, the macule expands asymmetrically to become a patch that has irregular contours (Figs. 17A and B). The range of color is usually less than that occurs in SSM. Typically, the lesions are a combination of tan...

Intraepidermal Vesicles

Primary infection usually occurs in childhood and may be subclinical with acute gingivostomatitis Recurrent lesions occur at a similar site each time, usually on the lips, face, or genitalia Initial infection results in a generalized vesicular eruption (chickenpox) Recurrent infection results in an acute, self-limiting, vesicular eruption occurring in a dermatomal distribution Vesicles become pustular, then form crusts that may lead to scarring Pain, tenderness, or paresthesias precede or...

Normal Histology

FORDYCE'S SPOTS AND MONTGOMERY'S TUBERCLES Groups of tiny white or yellow discrete papules (Fig. 2A) Vermilion border of the lips, particularly upper lip, genital skin, or on the oral mucosa In 70 to 80 of elderly persons Tiny slightly raised 1 to 2 mm papules on the areolae of breasts. Present in nearly every adult woman and sometimes in men A single sebaceous gland or sebaceous lobules situated high in the submucosa or dermis (Figs. 2B and C) Direct opening onto the surface (Fig. 2C)...

Microscopic Polyangiitis

Synonyms Microscopic polyarteritis renal-dermal vasculitis syndrome. Systemic neutrophilic small vessel vasculitis without extravascular granulomas or asthma Commonly associated with rapidly progressive renal disease (focal segmental necrotizing glomerulonephritis), skin involvement, and antibodies to pANCA (mostly MPO) (< 80 ) Palpable purpura and petechiae (> 75 ) (Fig. 15A) < 20 splinter hemorrhages (Fig. 15B), nodules, palmar erythema, and livedo Lack of biopsy or surrogate markers of...

Cutaneous Polyarteritis Nodosa

Synonyms Cutaneous periarteritis nodosa localized polyarteritis nodosa. Clinical Presentation (Fig. 16) Skin Manifestations Painful 0.5 cm to 2 cm lower to upper extremity nodules associated with livedo reticularis (Fig. 16C) Gangrene and digital necrosis, rare Atrophie blanche can be a manifestation in patients who have No evidence of venous insufficiency and thrombo-philia Signs of mononeuritis multiplex Benign course in most patients including children Patients with ulcers have more...

Lentigo Simplex

Lentigo simplex is a circumscribed, uniformly pigmented tan, brown, or dark brown macule usually 1 to 5 mm in diameter occurring either as a solitary lesion or as multiple lesions, possibly in the context of an inherited syndrome, anywhere on the cutaneous or mucocutaneous surface (Fig. 1). These lesions demonstrate regular and well-defined borders. Histo-logically, they show increased numbers of solitary basilar melanocytes and increased epidermal basal layer and possibly suprabasal layer...

Pernio

Synonyms Dermatitis congelationis chilblains perniosis erythema pernio. Papules, papulovesicles, nodules, and ulcerations Fingers, toes, nose, and ears Superficial and deep perivascular infiltrate of lymphocytes Edema of the papillary dermis Lymphocytes at the dermoepidermal junction often Thrombi in the lumen and or fibrin in the wall of vessels sometimes Mucin in the reticular dermis 1. Ackerman AB, Boer A, Bennin B, Gottlieb GJ. Histologic Diagnosis of Inflammatory Skin Diseases. 3rd ed. New...

Polymorphous Light Eruption

Synonyms Polymorphic light eruption summer prurigo, summer eruption prurigo aestivalis. Scattered edematous papules and plaques Sites exposed to sunlight, mostly the face, chest, and arms Variations include actinic prurigo (occurs in Indians of North and South America) and spring eruption of juveniles (vesicles on helices of boys) Sparse to moderately dense infiltrate of lymphocytes Extravasated erythrocytes often Marked edema of the papillary dermis Spongiosis of variable extent sometimes...

Proliferating Pilar Cyst

Synonym Proliferating trichilemmal cyst. Arise on scalp of females (90 ) Usually larger than an ordinary pilar cyst (Fig. 16A) May be more exophytic and ulcerated Tight feeling, but not painful Dermal cystic structure (Fig. 16B) Compressed, often appearing layered, epithelial lining Epithelial lining similar to pilar cyst with acanthosis, multiple layers of well differentiated squamous cells, swollen-appearing keratinocytes (Figs. 16C and D) Rare lesions may have a lining similar to an...

Benign Lichenoid Keratosis

Synonym Lichen planus-like keratosis. Chest, back, neck, upper extremities of adults over 40 Light brown to red, may notice brown at periphery Often associated with or arising in a solar lentigo (Fig. 12C) Variety of histologic subtypes including atypical and bullous Atrophic to hyperplastic epidermis Compact ortho- and parakeratosis Lichenoid, predominantly mononuclear cell infiltrate, obscures dermal-epidermal junction (Fig. 12D) Scattered necrotic keratinocytes May have mild keratinocytic...

Cutaneous Leukocytoclastic Angiitis

Synonyms Hypersensitivity vasculitis allergic vasculitis necrotizing vasculitis leukocytoclastic vasculitis primary cutaneous small vessel vasculitis. All ages, both sexes affected, mostly middle-aged adults Most cases idiopathic prolonged exercise, infectious or drug trigger in remainder Single or recurrent crops of palpable purpura affecting lower extremities Dependent areas of buttocks, lower legs, ankles and feet Areas of friction or constant pressure such as skin underlying belt, or sock...

Henoch Schonlein Purpura

Synonyms HSP Acute infantile hemorrhagic edema (putative variant). Most common vasculitis in children ( 90 all cases) affects adults as well Preceding upper respiratory tract infection occurs in up to 50 Recent drug or food ingestion triggers in minority Clinical tetrad skin involvement universal, other findings less frequent Retiform or patterned purpura and retiform margins considered specific to HSP Gastrointestinal hemorrhage (33 ) Features with high sensitivity and specificity for...

Pilar Cyst

Synonyms Wen trichilemmal cyst follicular cyst (isthmus- Common, occur most frequently on scalp, other areas with terminal hairs (Fig. 15A) Firm, round nodule, movable on palpation Except for site, indistinguishable from an epidermoid cyst May be red, tender, or painful when inflamed Intradermal (may extend into the fat) cyst, round or well circumscribed (Fig. 15B) Epithelial lining differs from normal epidermis, simulates isthmus portion of hair follicle Palisaded basal layer, one or two...

Histology

Exophytic, well circumscribed, symmetric (Fig. 1B) Variations of papillated epidermal hyperplasia (papillo-matosis) and thickening of the spinous layer (acanthosis) Interconnecting and bridging of the elongated rete ridges Numerous pseudohorn cysts created by cross-sectioning the keratin filled crypts between proliferations (Fig. 1C) Composed predominantly of small, uniform basaloid keratinocytes with a 1 1 to 1 0.5 nuclear to cytoplasmic ratio (Fig. 1D) Basalar keratinocytes are often...

Foreign Body Granulomas Allergic Sarcoidal Granulomatous Reaction

Nodular sarcoidal type of granulomatous disease. Some foreign bodies can induce an allergic granulo-matous reaction that simulates a sarcoidal granulomatous reaction. Granulomata secondary to silica (usually from glass or sand) can occur the foreign body can be detected as doubly refractile crystals with polarizing lenses. Beryllium (from cuts due to broken fluorescent light bulbs manufactured prior to 1950) results in granulomata often associated with central necrosis. Beryllium granuloma and...

Table 1 Clinical Criteria Used for the Classification of Benign Melanocytic Neoplasms

Age of onset congenital or acquired Size Small congenital nevus < 1.5 cm Medium sized congenital nevus > 1.5-20 cm Large congenital nevus > 20 cm Garment or bathing trunk nevus Segmental nevus Anatomic location Nonglabrous skin Glabrous acral Mucosal Genital flexural Other sites such as breast, scalp, ear, etc. Appearance Border characteristics (symmetry, circumscription) Surface topography (macular, papular, papillomatous, verrucoid) Pattern of coloration variegated or homogeneous Colors...

Definition of Terms

Follicular density Number of hair follicles per surface area obtained by counting the total number of follicles in a horizontally sectioned scalp biopsy and dividing by the area of the biopsy. (The area of a 4 mm punch biopsy is 12.57 mm2.) Normal follicular density in Caucasians is approximately 2 to 2.5 follicles mm2 and for African-Americans is 1.6 follicles mm2. Terminal to vellus (T V) ratio Obtained by counting the number of terminal hairs and dividing by the number of vellus hairs. This...

Contents

Desmoplastic Trichoepithelioma Inverted Follicular Keratosis Tumor of Follicular Infundibulum Fibrofolliculoma andTrichodiscoma Follicular tumors are one form of adnexal tumors. The adnexa in dermatopathology are skin appendages the hair follicles, sebaceous glands, and sweat glands. In gynecologic pathology, the adnexa are fallopian tubes and ovaries. Follicular, sebaceous and sweat gland tumors are not necessarily derived from such structures we prefer to think that immature pluripotential...

Granuloma Faciale

Nodular dermatitis with neutrophils, eosinophils, and plasma cells prominent. Solitary or several reddish brown nodules to plaques (Fig. 3A) Typically on the face although rarely extrafacial Persistent and asymptomatic Nodular mixed infiltrate with characteristic sparing of the papillary and periadnexal dermis (together known as the adventitial dermis) (Fig. 3B). Polymorphous infiltrate consisting of numerous neutrophils, many of them with fragmented nuclei (leukocytoclasis), eosinophils,...

Spongiotic Dermatitis

Spongiotic dermatitis is a histologic rather than a clinical diagnosis that is characterized by intraepidermal, intercellular edema (spongiosis) with or without microvesicle formation. Table 4 Differential Diagnosis Benign Familial Pemphigus Table 4 Differential Diagnosis Benign Familial Pemphigus

Viral Exanthems

Exanthem of macules and or papules Sometimes morbilliform (measles), and rubeoliform, (German measles) Variations include erythema infectiosum (appearance of cheeks that have been slapped), roseola exanthema subitum (discrete, small macules and papules similar to those of rubella) Sparse perivascular infiltrate of lymphocytes Extravasate erythrocytes, sometimes Sparse superficial perivascular infiltrate

Nodular Malignant Melanoma Clinical Presentation

All nodules of malignant melanoma begin as macules. The macules extend horizontally to form patches and then vertically to form papules and then nodules. The difference between a nodule of melanoma that develops in an SSM, an LMM, or an ALM and a nodular melanoma is that a nodular melanoma has no patch or plaque component detectable by inspection of the lesion. The nodules are mostly black in color with fairly uniform pigmentation (Fig. 15A). Nodular malignant melanomas (NMs) frequently are...

Severity Classification

Class I, mild disease Nodular skin lesions, livedo reticularis and or mild polyneuropathy Class II, severe disease Prominent livedo, ulcers, pain, polyneuropathy, and constitutional symptoms of fever, malaise, arthralgias Class III Progressive systemic disease necrotizing livedo, acral gangrene, foot drop, progressive musculo-skeletal involvement, positive autoimmune tests, and eventual visceral involvement Neutrophilic muscular vessel vasculitis occurring typically at the dermal-subcutis...

Histology Porphyria

Pauci-inflammatory subepidermal bulla with variable neutrophils, depending on the degree of necrosis (Fig. 12C) Festooning with preservation of dermal papillae on the floor of the blister, another diagnostic feature (Figs. 12C and D) Dramatic homogeneous eosinophilic PAS+ deposits around thickened elongated papillary dermal vessels a diagnostic feature (Figs. 12D and E) Caterpillar bodies (rows of dead keratinocytes in the epidermis) less often seen

Acute Cutaneous Leishmaniasis

Clinical Presentation After a bite by a sandfly, the patient develops a papule that grows into a nodule or plaque The nodule plaque ultimately will ulcerate (Fig. 25A) or become verrucous. Most resolve spontaneously with a scar Exposed areas are most commonly involved Most often solitary but can be multiple lesions

Dermal Melanocytoses

The dermal melanocytoses are lesions comprised of dermal dendritic melanocytes with a predilection for certain anatomic sites in persons of color. These conditions often appear at birth or shortly thereafter as gray bluish or brown patches. In some instances papular lesions resembling blue nevi may develop in the Nevi of Ota and Ito. Rare lesions may involve large segments of the skin sometimes with zosteriform distributions and some may develop later in life as acquired dermal melanocytoses.

Intracorneal Or Subcorneal Vesicle And Pustule

Synonyms Staphylococcal scalded skin syndrome (SSSS) Clinical Presentation SSSS (Fig. 1A) Primarily affects neonates in young children but may occur in adults, particularly if immunocompromised Sudden onset of malaise, fever, irritability, cutaneous tenderness, and erythema Erythema often accentuated in flexural and periorificial areas Flaccid blisters and erosions developed within one to two days Cultures of blisters are negative Sudden onset of macular erythroderma with desquamation of tips...

Erythrasma

Solitary patches and subtle red plaques covered by fine scales Coral-red fluorescence when exposed to Wood's light Elderly persons of both sexes, especially those with diabetes mellitus Sparse superficial perivascular infiltrate of lymphocytes Blue-staining organisms in the form of delicate rods and filaments in the cornified layer Gram stain shows delicate gram-positive rods and filaments in the cornified layer cornified layer, oriented horizontally

Sebaceoma

Synonym Sebaceous epithelioma (old term). Clinical Presentation Solitary yellowish circumscribed nodule or an ill-defined plaque (Fig. 10A) Usually on the face or scalp May be multiple in Muir-Torre syndrome Usually small size, symmetric, and well-circumscribed (Figs. 10B and 11A) Aggregations of neoplastic cells with great variation in size and shape (Figs. 10B and 11A) (1) Sebaceous lobules comprised of small, undifferentiated basaloid cells and mature sebocytes. The ratio of basaloid...

Clinical Presentation Fig 21A

Folliculitis that occurs on the occipital scalp and results in scarring alopecia Mainly affects African-American men, however, African-American women and occasionally other ethnic groups may develop the disease The earliest clinical lesions are follicular papules and occasional pustules With time, these papules become fibrotic with associated hair loss A small number of patients develop large scars resembling keloids on the nape

Classification Of Glandular Adnexal Neoplasms

Historically, adnexal neoplasms have been classified into four broad categories, namely follicular, sebaceous, apocrine, and eccrine. In light of the embryological considerations discussed previously, a logical ontogenetic classification yields but two (folliculosebaceous-apocrine and eccrine). This condensation is of no consequence for an established entity with a singular line of differentiation, such as sebaceous adenoma. The classification schemes of the past placed sebaceous adenoma as a...

Clinical Features

Clinically atypical melanocytic nevus. Increased numbers of typical and atypical nevi (e.g., > 50 or 100) Variation in gross morphologic features among nevi Increased numbers of nevi on scalp, female breasts, and buttocks Increased size (4 to 12 mm usually but exceptions) Altered topography, pebbled or cobblestone surface Haphazard, variegated or greater complexity of coloration

Tineaversicolor

Synonyms Pityriasis versicolor dermatomycosis furfuracea. Slightly scaly macules and patches Hypopigmented in dark-skinned and hyperpigmented in light-skinned persons Symmetrical on the trunk, sometimes involving proximal extremities Sparse superficial perivascular infiltrate of lymphocytes Short branching septate hyphae and spores in the corni-fied layer Slight hyperkeratosis in basket-weave fashion Slight spongiosis and parakeratosis rarely Malassezia furfur in the cornified layer

Cutaneous Inflammatory Pseudotumor

Solitary cutaneous nodule or nodules with histologic features that mimic either erythema elevatum diutinum or granuloma faciale histologically, but not clinically Pathogenesis is believed to be due to either systemic or local immune complexes producing recurrent vessel damage to the small, easily injured vessels of fibrosing granulation tissue All three disorders are characterized by small vessel neutrophilic vasculitis, patterned fibrosis, and dense nodular mixed, neutrophilic rich...

Pseudolymphoma

Nodular dermatitis consisting predominantly of lymphocytes. Synonyms Cutaneous lymphoid hyperplasia B- or T-cell lymphoid hyperplasia lymphadenosis benigna cutis Commonly involves head, neck, and upper trunk Usually asymptomatic red-brown papules or nodules (Fig. 1A) Usually single, may be multiple, rarely widespread Nodular to diffuse infiltrates of lymphocytes throughout the dermis and subcutaneous tissue (Fig. 1B) Polymorphous infiltrate usually (lymphocytes, plasma cells, histiocytes,...

Extraocular Sebaceous Carcinoma

Usually on the head and neck of elderly patients (2) Yellowish firm nodules, often ulcerated, 1 to 4 cm or more in diameter (Fig. 12A) Located superficially or deep (Figs. 12B and 13A) Asymmetric lesion with infiltrative borders (Fig. 13A) Neoplastic aggregates with variation in sizes and shapes bear little resemblance to normal sebaceous lobules (Figs. 12B 13A, B, and C) (1,2) Mixture of immature and mature neoplastic sebocytes (Figs. 12C and D 13B, C, and D) Markedly pleomorphic neoplastic...

Classification of Panniculitis Primary Panniculitis

Panniculitis refers to inflammation of the subcutaneous tissue. This chapter will focus on common and unusual types of noninfectious primary panniculitis and review several examples of secondary panniculitis that may be due to multiple etiologies. In most cases of panniculitis, special stains should be performed to exclude an infectious etiology polarized light examination should also be performed to identify foreign material. Clinical evaluation and directed laboratory studies may be necessary...

Adnexal Carcinoma

Adnexal carcinomas (adenocarcinomas) are relatively uncommon, and their rarity has contributed to confusion with respect to diagnosis, classification, and therapy. Because of their infrequency, description of adnexal carcinomas has often come in the form of case reports, and large series that could serve as the foundation for lucid conclusions regarding behavior and therapy has been difficult for investigators to assemble. The literature is probably also skewed by inclusion of extraordinary...

Nodular Vasculitiserythema Induratum Of Bazin

Clinical Presentation Predilection for middle-aged females Ulceration and atrophic scarring are common (Fig. 8A) Protracted and recurrent episodes over years Most frequently located on calf (Fig. 8A) Erythrocyanosis, column-like calves, erythema surrounding follicular pores, cutis marmorata Hypersensitivity reaction precipitated by tuberculosis erythema induratum

Histology Figs 14AD

Band-like lymphocytic infiltrate obscuring the interface between the follicular epithelium and the dermis, with associated necrotic keratinocytes and occasionally formation of colloid bodies. Inflammation is most dense at the level of the infundi-bula and isthmus, but can extend around the inferior portions of follicles. Early disease demonstrates just the lymphocytic inflammation, without loss of follicles or sebaceous glands. Well-developed lesions demonstrate concentric perifollicular...

Other Intracytoplasmic Organisms

capsulatum (fungus) Rhinoscleroma Klebsiella rhinoscleromatis (gram-negative bacillus) Granuloma inguinale-Calymmatobacterium granulomatis gram-negative bacillus (Donovan body). 1. Hepburn MC. Cutaneous leishmaniasis. Clin Exp Dermatol 2000 25 363-370. 2. Grevlink SA, Lerner EA. Leishmaniasis. J M Acad Dermatol 1996 34 257-272. Diffuse dermatitis with prominent mast cells. Orange-brown macules papules and nodules on trunk less often extremities (Fig. 8A) Lesions develop wheal and flare...

Pemphigus Foliaceus And Pemphigus Erythematosus

Synonyms Pemphigus foliaceus superficial pemphigus, fogo selvagem (endemic form in South America) pemphigus erythematosus Senear-Usher syndrome. Clinical Presentation Pemphigus Foliaceus (Fig. 2A) Recurrent shallow erosions, erythema, scaling, and crusting Small flaccid blisters that rupture easily Mucous membrane involvement uncommon Circumscribed patches of erythema and crusting, that are localized to nose, cheeks, and ears, and that resemble lesions of lupus erythematosus Crusting and bullae...

Secondary Syphilis

Clinical Presentation Syphilis is an infectious, sexually-transmitted disease, the clinical manifestations of which are protean secondary syphilis is known as the great imitator, producing cutaneous lesions of every morphology except pustules Secondary syphilis generally presents 2 to 10 weeks after the appearance of the painless ulcer (the chancre of primary syphilis) Patients with secondary syphilis show a generalized lymphadenopathy associated with mucocutaneous lesions that...

Lichenoid Drug Eruption

Characterized by the development of erythematous-to-violaceous, flat-topped papules, and plaques on the trunk and extremities after ingestion of a drug Most commonly implicated drugs are gold, -adrenergic antagonists, captopril, penicillamine, and antimalarials The eruption clears slowly several weeks after discontinuation of the offending agent. Focal parakeratosis (Fig. 9B) Wedge-shaped hypergranulosis (Fig. 9A) Jagged, saw-tooth acanthosis of the epidermis (Fig. 9A) Band-like, mixed...

The Clinically And Histologically Atypical Melanocytic Nevi The Socalled Dysplastic Nevus

For almost thirty years melanocytic nevi seeming to occupy an intermediate position between common banal nevi and melanoma have remained controversial as to their biological nature, significance, and nomenclature. Although considerable research has been conducted on the subject and new important information has emerged, the inability to develop minimal essential clinical and histological criteria for such atypical nevi has encumbered the generation of clean data. Furthermore, it seems likely...

Epidermoid Cyst

Synonyms Keratin cyst epidermal inclusion cyst sebaceous cyst follicular cyst (infundibular type). Extremely common, face, scalp, trunk, extremities Slow growing, round, firm, intradermal tumors (Fig. 14A) Normal skin surface, usually May have overlying pore or dell May be red, tender, or painful when inflamed Milia are very small cysts, usually on the face, often derived from vellus hairs Intradermal (may extend into fat) cystic structure (Fig. 14B), round to oval, well circumscribed True...

Benign Familial Pemphigus

Clinical Presentation Eczematous Dermatitis Eczematous dermatitis is a general term for a pruritic rash composed of minute papules and intraepidermal vesicles. Common types of eczematous dermatitis include Contact dermatitis (allergic and irritant) (Fig. 7A) Geometric or linear configuration Conforms to area of contact Chronic pruritic disease that begins in childhood and follows the remitting recurrent course that may continue through life Occurs in patients with the personal or family history...

Desmoplastic Melanoma Clinical Presentation

Desmoplastic melanoma (DM) is a type of spindle cell melanoma that stimulates fibroplasia in the dermis. The incidence of DMs is not known with certainty, but they comprise only a few percent of all melanomas. DM may develop at many anatomic sites, but most commonly, they are situated on the head and neck region. These lesions usually lack pigmentation and may be mistaken for nonmelanoma skin cancers or scars (Fig. 26). They present as firm papules, plaques, or nodules. Although usually...

Histology Figs 16AC

Interface changes at the dermal-epidermal junction and the basement zone region of follicular epithelium Necrotic keratinocytes and lymphocytic inflammation are present in these interface areas There is superficial and deep perivascular, perifollicular, and follicular inflammation consisting of lymphocytes and plasma cells Prominent hyperkeratosis overlying follicular infundi-bula The follicles are eventually replaced by fibrosis (hair granulomas may be present) and there may be...

Genitalflexural Nevi

These variants of common acquired nevi are largely defined by particular clinical and histological features related to anatomic site, whether this is genitalia (vulva, perineum, and male genitalia), or umbilicus, and other flexural sites such as the axillae. Such nevi may be slightly larger in size and may share some clinical and histological characteristics with other atypical (dysplastic) nevi such as architectural disorder and cytological atypia (Table 10) (Fig. 6). The relationship of this...

Benign Neoplasms

SEBACEOUS ADENOMA Clinical Presentation Flesh-colored or yellow solitary circumscribed nodule (Fig. 8A) Head and neck, especially face or scalp Usually < 1 cm in diameter Sharply demarcated, mainly endophytic neoplasm (Figs. 8B and 9A) (1) Large sebaceous lobules with irregular size and shape (Figs. 8B and D 9B) Sebaceous lobules oriented vertically, which either connect directly to the skin surface or lead to channels that serve both as sebaceous duct and infundibulum (Figs. 8C 9C and D) (1)...

Hair Nevus

Synonym Vellus hamartoma may be the same thing as an accessory tragus in some cases. Exceedingly rare, onset at birth through young adulthood Often dome-shaped papule or plaque Localized proliferation of hairs Increased numbers of mature hair follicles. Usually these are vellus hairs, except in the nevus pilosus variant in the scalp where terminal hairs are found May be associated with basal layer hyperpigmentation (lentigo-like changes in Becker's nevus) or with increased smooth muscle bundles...

Melanocytic Neoplasms Neoplasms

Location of melanocytes in the skin (depth) Superficial Intraepidermal Papillary dermis Upper half of reticular dermis Deep Lower half of reticular dermis Subcutaneous Fascial Disposition of melanocytes Intraepidermal Basilar melanocytes (single cell pattern) Normal numbers Increased frequency - With elongated rete (lentiginous) - Without elongated rete Pagetoid pattern Patchy perivascular, periadnexal, perineurial Wedge pattern (deep apex of nests, fascicles of melanocytes extend into...

Classification and Criteria for Benign Melanocytic Neoplasms

Benign melanocytic neoplasms constitute a heterogenous spectrum of lesions that are classified according to a number of clinical, histological, and other attributes (Tables 1 and 2). As with any classification there is controversy as to the basis for defining and including the various entities in such a classification. The scheme outlined in Table 3 will be utilized in this chapter. Major considerations for classification include age of onset of the lesion, size, anatomic site, other gross...

Clear Cell Acanthoma

Synonym Degos acanthoma Clinical Presentation Solitary, rarely multiple, usually on lower legs of adults Well circumscribed papule or small plaque (Fig. 10A) May have shiny or moist appearing surface Often with a peripheral collarette of scale Circumscribed zone of acanthosis Proliferation of keratinocytes with normal nuclei and clear or pale staining cytoplasms filled with glycogen (Fig. 10B) Sharp circumscription and sparing of adnexal epithelium (Fig. 10C) Neutrophils or neutrophil particles...

Actinic Keratosis

Synonyms Solar keratosis senile keratosis. Clinical Presentation Most commonly found on sun-exposed areas of fair-skinned individuals in middle- or later-age groups Remarkable variance in clinical and pathologic characteristics An isolated scaly plaque with an erythematous base (Fig. 1A) Few millimeters in diameter to rarely a plaque a few centimeters in diameter Often multiple (Fig. 1B) and widely or locally distributed May appear as papulonodules, atrophic plaques, or with cutaneous horns...

Epidermolysis Bullosa Simplex

Synonyms Koebner generalized form of epidermolysis bullosa simplex (EBS) Weber-Cockayne (localized form of EBS). Clinical Presentation (Fig. 12A) Koebner (Generalized Form of EBS) Occurs at birth or shortly thereafter may improve with time Vesicles, bullae, and milia over the joints of the hands, elbows, knees, feet, and other sites subject to repeated trauma Lesions are sparse and do not lead to atrophy or severe scarring Worse during summer improves during winter

Stucco Keratosis

Symmetric distribution on lower legs Small, 1 to 4 mm, hyperkeratotic papules with adherent horn (Fig. 6C) Light tan, uniform in color Variant of seborrheic keratosis Church spire digitations, mild acanthosis (Fig. 6D) INVERTED FOLLICULAR KERATOSIS Clinical Presentation Usually on face or scalp of elderly patients (Fig. 7A) 2 to 10 mm papules with central scale Difficult to distinguish from a seborrheic keratosis or verruca vulgaris Well circumscribed and symmetric Proliferation of bland...

Synonym Tuberculosis luposa Clinical Presentation

The most common variant of cutaneous tuberculosis presents as reddish brown papules, nodules, or plaques (Fig. 3A) that have an apple jelly color especially noted on diascopy. As the lesions expand, they often are associated with central scarring that can cause significant tissue destruction. Aggregates of epithelioid histiocytes generally in the upper half of the dermis surrounded by a mantle of lymphocytes (Figs. 3B and D) and associated with overlying epidermal thinning (rather than...

Other Clinical Patterns

Ophiasis hair loss (bands of hair loss at the scalp margins) (Fig. 1B) inverse ophiasis pattern (loss of hair on the crown with retention of hair along the scalp margins) alopecia totalis (loss of all scalp hair) alopecia universalis (loss of all scalp and body hair) diffuse alopecia areata (diffuse thinning without discrete areas of hair loss, although tiny discrete areas can sometimes be found on careful examination) (Fig. 1C) Exclamation point hairs (short broken-off hairs that taper...

Synonym Hansens disease Clinical Presentation

One to just a few asymmetrically scattered hypopigmented well demarcated anesthetic plaques (Fig. 4A) Occasionally, plaques may demonstrate erythema, central clearing, and more peripheral induration Elliptical aggregates of epithelioid histiocytes surrounded by lymphocytes, multinucleated histiocytes, and occasional plasma cells especially in the lower dermis (Figs. 4B and E). Granulomas can be identified around small nerves, hair erector muscles, follicles, and sweat glands (Figs. 4C-G)...

Rosacea And Perioral Dermatitis

Nodular tuberculoid type of granulomatous disease. Red to red-brown follicular papules and pustules on the face (Fig. 6A) Aggregates of epithelioid histiocytes surrounded by lymphocytes usually around or adjacent to hair follicles (Figs. 6B and C) Necrosis can be identified within the granulmata Starts as a suppurative follilculitis progresses to a granulomatous folliculitis

Interface Dermatitis

SUNY-Downstate Medical Center, Department of Dermatology, Brooklyn, New York, U.S.A. --VACUOLAR INTERFACE DERMATITIS SUPERFICIAL VACUOLAR INTERFACE DERMATITIS ERYTHEMA MULTIFORME Vacuolar Interface Dermatitis Superficial Acute Graft-vs.-Host Reaction Systemic Lupus Erythematosus Lichen Sclerosus and Atrophicus Vacuolar Interface Dermatitis Superficial and Deep Discoid Lupus Erythematosus Pityriasis Lichenoides and Varioliformis Acuta LICHENOID INTERFACE DERMATITIS Lichenoid Interface Dermatitis...

Clinicopathologic Correlation

Indurated plaque on extremity with overlying hyperkeratotic scale and follicular plugging Hyperkeratosis vacuolar interface dermatitis basement membrane thickening superficial and deep perivascular and periadnexal inflammation with dermal mucin Lobular and septal panniculitis with lymphocytes, plasma cells, and hyaline fat necrosis

Clinical Presentation Fig 19A

A form of CCSA characterized by the presence of numerous pustules at the periphery of the scarred area(s) The lesions may be quite painful and patients often complain of drainage and or bleeding on the pillow at night when the disease is active More common in men, reported in Caucasians and African-Americans Tufted folliculitis, characterized by several (up to 30) terminal hairs emerging from the same follicular orifice in the center of an area of scarring, is often seen in patients with...

Pseudovasculitis

As the clinical manifestations of vasculitis are protean, and diagnosis is based on a constellation of clinical, histologic, imaging and laboratory features, it is not surprising that nonvasculitis disorders can mimic vasculitis. Many of these pseudovasculitic disorders will involve dermal hemorrhage and or occlusion of vessels with infarction by emboli, thrombi, vasospasm, fibro-intimal-medial hyperpla-sia secondary to vessel trauma, or noninflammatory vessel wall pathology such as...

Spitz Tumor With Atypical Features

A subset of Spitz tumors may be difficult or impossible to distinguish from melanoma and may exhibit one or more atypical features such as large size (often > 10 mm), asymmetry, poor circumscription, ulceration, deep involvement, high cellular density, confluent or nodular grow patterns, diminished or absent maturation, cytological atypia beyond what is acceptable for a Spitz tumor, and significant dermal mitotic rates including deep or marginal mitoses (Tables 13 and 14) (Fig. 10). The...

Squamous Cell Carcinoma In Situ

Squamous cell carcinoma in situ (SCCIS) is a histologic term with many diverse clinical presentations. The biologic importance is also variable from a benign course in bowe-noid papulosis to possible invasive growth potential in Bowen's disease. It is extremely important to make a clinico-pathologic correlation (CPC) when a histologic diagnosis of SCCIS is made. For example, bowenoid papulosis has the histologic features of SCCIS, which is considered to be a benign, virally induced condition....

Antigens

Hemidesmosomal proteins BPAgl (230 kDa) and BPAg2 (180 kDa, which is also known as collagen XVII) IgG antibodies bind to BP antigens and activate C3 and inflammatory mediators. Eotaxin, a chemokine, may recruit eosinophils to the basement membrane zone. Inflammatory cells release proteases at the basement membrane zone, and hemidesmosomal proteins are degraded, leading to blister formation. No epidermal changes stratum corneum

Bullous Drug Eruption

Bullous pemphigoid Pemphigoid gestationis Mucous membrane pemphigoid Linear IgA bullous dermatosis Salt Split Skin Separation Anchoring filaments Epidermolysis bullosa acquisita Bullous lupus erythematosus Bullous pemphigoid Pemphigoid gestationis Mucous membrane pemphigoid Linear IgA bullous dermatosis Salt Split Skin Separation Anchoring filaments Epidermolysis bullosa acquisita Bullous lupus erythematosus Figure 1 Schematic of the basement membrane zone antigens recognized by autoantibodies...

Cellular Blue Nevus

Cellular blue nevi (CBN) clearly occupy a continuum with the more prevalent common BN. CBN are often larger lesions commonly exhibiting a so called biphasic morphological configuration (Fig. 13A). The latter refers to the presence of a common BN component often superficial that shows transition to a deeper multinodular cellular component (Fig. 13B). The principal feature of this deeper component is fairly discrete bundles, concentric aggregates, or sometimes broad sheets of spindled melanocytes...

Pemphigoid Gestationis

Clinical PG is a blistering disorder of young women during late pregnancy (last trimester) or in the early postpartum period. It can be exacerbated by oral contraceptives and by repeat pregnancy with same partner PG is characterized clinically by urticarial plaques or wheals that evolve to vesicles and bullae (Fig. 4A). The eruption begins in the periumbilical area. About 5 of neonates born to mothers with pemphigoid gestationis may develop transient blisters....

Ocular Sebaceous Carcinoma

One percent of all eyelid neoplasms Often masquerades as an inflammatory disease (3) Slight female preponderance Upper eyelid more commonly involved than lower eyelid (1,3) Up to one-third develop lymph node metastases Twenty percent five-year mortality Usually superficial location Asymmetry, poor circumscription Aggregations of neoplastic sebocytes vary in sizes and shapes (1,3) Maturation of neoplastic sebocytes varies from immature (nonvacuolated) to mature (vacuolated) Nuclei of neoplastic...

Warty Dyskeratoma

Not a true acanthoma, but may simulate both clinically and histologically. Solitary papule on face, scalp, or neck Slightly umbilicated or depressed, keratin filled center Central invagination, cup-shaped (Fig. 13C) May demonstrate association with the infundibulum of a hair follicle Acantholytic and dyskeratotic keratinocytes, often in papillary projections (Fig. 3D) May have villous pattern with normal basaloid cell layer, progressing to acantholysis and dyskeratosis

Tubulopapillary Papillary Adenoma andAdenocarcinoma

The nosology of adnexal neoplasms has been confused and confusing for decades, and much of the mystification of the past was wrought by the lack of logical classification. Classification proposals and inferences regarding lineage from the authorities of the past were often contradictory, and to a lesser extent, this problem persists at the present time. This is in part a consequence of the fact that broad conclusions regarding lineage and classification were based on enzyme histochemical...

Lichenoid Interface Dermatitis

Dense, band-like inflammatory cell infiltrate, which obscures the dermoepidermal junction Infiltrate may be only in the superficial dermis, or both the superficial and deep dermis LICHENOID INTERFACE DERMATITIS SUPERFICIAL LICHEN PLANUS Clinical Presentation Chronic dermatosis with skin, hair follicle, and mucous membrane involvement 10 of cases show involvement of the nail unit, often with pterygium formation Presents clinically as pruritic, violaceous, flat-topped, polygonal papules, and...

Superficial Spreading Melanoma Clinical Presentation

SSM occurs most commonly on the back and shoulders in men and on the legs or back in women. SSM begins as a small, brown, asymmetric macule. As it grows within the epidermis, it expands centrifugally, but it does not progress at the same rate in all directions, which results in variegation in color and more prominent asymmetry (Figs. 11A and B). The color may range from black to brown to blue to pink in different parts of the lesion. If regression occurs, the involved focus may appear gray or...

Verruca Vulgaris

Verrucae often simulate seborrheic keratoses, both clinically and histologically. Common on face, hands, may occur at any site Hyperkeratotic with velvety surface, smooth if rubbed (Fig. 8A) Skin color to pink, no pigment Digitated or spiking epidermal hyperplasia (Fig. 8B) Koilocytes in the superficial layer of the epidermis Columns of parakeratosis and or serum above the tips of digitations Dilated blood vessels in the dermal papillae See Clinicopathologic Correlation (p. 173). See...

Xanthogranuloma

Clinical Presentation Tan to pink to red-brown, to yellow to yellow-orange papules, plaques or nodules. More yellow coloration noted in fully-developed lesions (Fig. 22A) Can present as many small nodules or one or a few large nodules Rare in adults, where it is usually a solitary persistent lesion In children often multiple and usually spontaneously resolve In infants and children can be associated with extra-cutaneous lesions, particularly ocular lesions...

Muirtorre Syndrome And Its Cutaneous Manifestations

Syndrome characterized by the development of sebaceous tumors, often multiple (Fig. 14A), and visceral neoplasms Autosomal dominant inheritance Sebaceous tumors vary from just one to more than 100 lesions Cutaneous tumors may precede or follow the manifestation of the visceral cancer (1) Visceral tumors usually of the gastrointestinal tract polyps of the large bowel and adenocarcinomas Other organs may be involved larynx, the genito-urinary system, ovary, and uterus Cutaneous sebaceous...

Acne Keloidalis Nuchae Also Known As Folliculitis Keloidalis

Figure 1 (A) Well-circumscribed areas of nonscarring hair loss in typical alopecia areata. (B) Marginal hair loss in ophiasis pattern alopecia areata. (C) Diffuse hair loss in diffuse alopecia areata. (D) Exclamation point hairs (arrows) in an active patch of alopecia areata. Figure 1 (A) Well-circumscribed areas of nonscarring hair loss in typical alopecia areata. (B) Marginal hair loss in ophiasis pattern alopecia areata. (C) Diffuse hair loss in diffuse alopecia areata. (D) Exclamation point...

Rare And Unusual Cysts

These lesions are cystic and clinically recognized only by their unusual locations. The diagnoses depend on multiple specific criteria with the type of epithelial lining being most important. Clinical Presentation and Histology Pigmented Follicular Cyst Brown cystic lesion, face and neck, may resemble other pigmented neoplasms, usually solitary Epithelial lining similar to normal epidermis of the infundibulum of a hair follicle Amorphous pigmented keratin in cavity, with or without portions of...

Follicular Diseases Causing Scarring Alopecia

Follicular destruction results in scarring alopecia that can be classified as primary or secondary. In primary scarring alopecias, the follicle is the target of inflammation. In secondary scarring alopecias, the follicle is an innocent bystander that, nevertheless, is destroyed. Examples of secondary scarring alopecias include morphea and tumors (alopecia neoplastica). In this chapter, we will consider only the primary scarring alopecias. In this group of diseases, the inflammation can be...

Basal Cell Carcinoma

Verrucous Carcinoma The Penis

Occurs in sites chronically exposed to the sun and increased in frequency from the third decade onward, although they have been reported in children and young adults. Most frequently on the face and scalp, rarely arises on the digits, the back of the hand, dorsum of the foot, or on the ears. Typically are solitary opalescent nodules with telangiectasia (Fig. 18A) or ulceration (Fig. 18B). Common variant is the superficial type (Fig. 18C), which often appears as a thin plaque. Morphea-form may...

Digital Myxoid Cyst

Most common on the dorsal surface of the index or middle finger, overlying the distal interphalangeal joint, just above the proximal nail fold (Fig. 20C) Ill-defined collection of mucin in superficial dermis, may have overlying collarette of epidermis (Fig. 20D) Mild inflammation at the periphery Figure 1 (A) Multiple well circumscribed, hyperplgmented, verrucous plaques with a stuck-on appearance. (B) Well circumscribed and symmetric lesion demonstrating papillomatosis and acanthosis of the...

Rl81a85434 2007

Visit the Informa Web site at www.informa.com and the Informa Healthcare Web site at www.informahealthcare.com This book is dedicated to the memory of my Dad, George H. Grant, D.D.S. who died on June 8, 2006 at noon and to my husband, Barry D. Kels, M.D., J.D. They are both gentle yet strong, demonstrate an absolute love of life and family, and my greatest supporters. In their arms, I have learned what it means to feel safe and secure. My Dad was the first man in my life that I loved with every...

Combined Nevus

Combined nevus is a variant of benign melanocytic neoplasm characterized by the presence of two or more distinct populations of melanocytes Table 21 Fig. 14 . Virtually, any combination of melanocytic components may occur, for example, common acquired nevus and common blue nevus, common nevus and pigmented spindle cell epithelioid cell dermal component Figs. 14B-D , Spitz and blue nevus components, congenital nevus and blue nevus and so on. Synonym Melanocytic nevus with phenotypic...

Clinical Evolution of Melanoma

Malignant melanomas may arise de novo, that is, in apparently normal skin or in association with a pre-existing melanocytic nevus. When malignant melanomas arise de novo, they begin as a small, lightly pigmented macule that in time is characterized by asymmetry, scalloped borders, poor circumscription, and variations in color of predominantly tan to brown Fig. 1A . Some such macular lesions become patches that are increasingly asymmetrical, poorly circumscribed, and varied in color Fig. 1B ....

Pigmented Spindle Cell Melanocytic Tumor

The pigmented spindle cell tumor PSCT is considered by some to be a pigmented variant of Spitz tumor, and it is clear that clinical and histological overlap occurs between the two lesions. However, the PSCT may show rather distinctive attributes in a large proportion of cases. The lesion usually presents as a small well-defined dark brown to black flat topped papule Fig. 11A . Histologically, PSCT is a sharply circumscribed mainly intraepidermal proliferation of small heavily pigmen-ted...