Dermatopathology Workshop Summary, Berlin 2004

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1 Dermatopathology Workshop Summary, Berlin 2004 David A. Whiting and Rolf Hoffmannw Baylor Hair Research and Treatment Center, Dallas, Texas, USA; wdermatology Practice, Freiburg, Germany Figure 1 Case 1: 42-y-old white female. Diffuse scalp hair loss for y(a, b). Chronic telogen effluvium: Normal numbers of terminal hairs, vellus hairs, and follicular stelae (streamers). Increased numbers of telogen hairs in active telogen effluvium. Mild to moderate, perifollicular, lymphohistiocytic inflammation in 40% of cases, as in normal hair. Figure 2 Case 2: 65-y-old white female. Diffuse hair loss from crown for 10 y (a, b). Female pattern alopecia: Increased miniaturized vellus-like hairs, telogen hairs, and follicular stelae; decreased terminal hairs. Mild to moderate perifollicular lymphohistiocytic inflammation in 70% of cases. Intact sebaceous glands. Copyright r 2005 by The Society for Investigative Dermatology, Inc. 256

2 10 : 3 DECEMBER 2005 DERMATOLOGY WORKSHOP, BERLIN 257 Figure 3 Case 3: 31-y-old white female. Patchy hair loss with scarring for 1 y (a, b). Lichen planopilaris: Peri-infundibular hypergranulosis may be seen. Interface alteration at follicle primarily lichenoid; pigment incontinence; moderate to prominent dyskeratosis. Lymphocytic inflammation perifollicular but not perieccrine. Absent epidermal and dermal mucin. Concentric lamellar fibrosis in advanced lesions. Figure 4 Case 4: 50-y-old white female. Patchy hair loss for 1 y; excoriations (a, b). Traumatic alopecia: Normal or reduced follicular counts, normal terminal to vellus hair ratio. Increased telogen and catagen hairs. Inflammation only increased if secondary infection present; fibrosis only increased by infection and chronic traction. Figure 5 Case 5: 43-y-old white female. Patchy scalp hair loss for 2 mo (a, b). Alopecia areata: Acute stage: Lymphocytes involve terminal bulbs in early and vellus bulbs in repeat episodes. Subacute stage: Less anagen, more catagen, and telogen hairs and stelae. Chronic stage: Less terminal hairs, more miniaturized hairs, and stelae; some inflammation. Recovery stage: More terminal hairs, less stelae, and no inflammation.

3 258 WHITING AND HOFFMAN JID SYMPOSIUM PROCEEDINGS Figure 6 Case 6: 53-y-old black female. Spreading scarring on vertex for 2 y (a, b). Central centrifugal cicatricial alopecia: Perifollicular inflammation of lower infundibulum and upper isthmus. May be associated with premature desquamation of inner root sheath in some cases. Progressive thinning of external root sheath leads to follicular distention, rupture, hair granulomas, and dense fibrosis. Figure 7 Case 7: 17-y-old black male. Recurrent abscesses on scalp for 2 y (a, b). Dissecting folliculitis: Follicular dilatation, abscess, rupture, and sinus tract formation with fibrosis. Inflammation with neutrophils, lymphocytes, histiocytes, plasma cells, and foreign body giant cells, initially perifollicular, but later dissecting through subcutis below hair bulbs. Figure 8 Case 8: 10-y-old black female. Patchy hair loss with pustules, 3 mo (a, b). Tinea capitis: Follicular dilatation, abscess formation, and rupture. Initial neutrophilic inflammation, later granulomatous with lymphocytes, histiocytes, plasma cells, eosinophils and foreign body giant cells; peri-infundibular, may extend to involve entire follicle. PAS-positive endothrix fungal spores and hyphae.

4 10 : 3 DECEMBER 2005 DERMATOLOGY WORKSHOP, BERLIN 259 Figure 9 Case 9: 26-y-old black male. Scarring folliculitis, occiput, 4 y (a, b). Acne keloidalis: Initial neutrophilic and later lymphocytic folliculitis caused dilatation and follicular rupture, causing hair granulomas and dense dermal fibrosis. Ingrowing hairs are not seen. Figure 10 Case 10: 20-y-old white female. Patchy hair loss on and off, 3 y (a, b). Trichotillomania: Normal follicular counts with normal terminal:vellus ratio. Marked increase in catagen and telogen hairs. Follicular damage may be present. Follicles may contain pigmented casts. Trichomalacia can occur. Inflammatory changes usually absent. Figure 11 Case 11: 59-y-old white female. Small red scaly lesions on the scalp, back, and arms for 8 mo (a, b). Discoid lupus erythematosus: Vacuolized basal layer, with dyskeratosis and pigment incontinence. Superficial and deep perivascular lymphocytes. Prominent peri and interfollicular and perieccrine lymphocytic infiltrates with dermal mucin. Perifollicular fibrosis in end stage.

5 260 WHITING AND HOFFMAN JID SYMPOSIUM PROCEEDINGS Figure 12 Case 12: 39-y-old white male. Several scalp tumors for 5 mo (a, b). Non-Hodgkin s lymphoma: Atypical perifollicular infiltration of dense lymphocytes, which vary in size and shape. Moderate fibrosis. Positive CD3 and CD20 stains indicate predominant B cell lymphoma. Figure 13 Case 13: 44-y-old Hispanic male. Recurrent folliculitis of scalp for 15 y (a, b). Tufted folliculitis: Acute and chronic inflammation led to ruptured follicles, polytrichia, and scarring. Causes include folliculitis decalvans, acne keloid, kerion, lichen planopilaris, discoid lupus erythematosus and central centrifugal cicatricial alopecia. Figure 14 Case 14: 31-y-old black male. Folliculitis, hair loss scalp/neck, 11 y (a, b). Folliculitis decalvans: Acneiform follicular dilatation. Follicular rupture and abscess formation. Inflammation initially neutrophilic, later lymphohistiocytic with plasma cells and foreign body giant cells, primarily peri-infundibular; it may extend to involve entire follicle. Perifollicular fibrosis is the endpoint.

6 10 : 3 DECEMBER 2005 DERMATOLOGY WORKSHOP, BERLIN 261 Figure 15 Case 15: 72-y-old black female. Receding frontal hairline and thinning eyebrows for 1 y (a, b). Frontal fibrosing alopecia: Slowly progressive loss of hair follicles due to lymphohistiocytic inflammation and lamellar fibrosis around lower infundibulum and isthmus. Many follicular units fibrose. May have features of lichen planopilaris. Figure 16 Case 16: 46-y-old black female. Nodules on the scalp, face, and arms with bone pain, 3 y (a, b). Sarcoidosis: Many perifollicular and interfollicular naked epithelioid cell granulomas. Relatively few lymphocytes and histiocytes but foreign body giant cells are seen. Acid fast and GMS stains negative. Figure 17 Case 17: 40-y-old white female. Itchy, scaly, excoriated, septic scalp, 4 y (a, b). Langerhans cell histiocytosis: Cell infiltrates lichenoid and epidermotropic or perivascular in papillary and reticular dermis, eosinophils. Tumor cells: abundant eosinophilic or pale cytoplasm; reniform or longitudinally grooved nuclei; small nucleoli. S-100 and CD1a (OKT6) positive.

7 262 WHITING AND HOFFMAN JID SYMPOSIUM PROCEEDINGS Figure 18 Case 18: 27-y-old white male: Patchy scalp hair loss (a, b). Alopecia mucinosa: Reticular degeneration of epithelial cells in the outer root sheath and sebaceous glands. Mucin initially deposited in the infundibulum, but can later involve the entire follicle. Mucicarmine stain faintly positive. Benign form here shows a slight lymphocytic infiltrate. Figure 19 Case 19: 51-y-old white male. Scarring alopecia on scalp, 1 y. (a, b). Nonspecific cicatricial alopecia: Lymphocytic infiltrate around the upper and mid-follicle. No interfollicular infiltrate. Loss of sebaceous epithelium. Follicular fusion. Moderate perifollicular lamellar fibrosis. Possible causes include lichen planopilaris. Figure 20 Case 20: 29-year-old white female. Universal hair loss since birth (a, b). Congenital hypotrichosis: Reduced terminal hairs. Reduced vellus hairs. No sebaceous glands. Cystic lesions typical of papular atrichia not present. Possible genes involved include hairless and vitamin D receptor genes.

8 10 : 3 DECEMBER 2005 DERMATOLOGY WORKSHOP, BERLIN 263 DOI: /j x Submitted September 20, 2004; accepted for publication September 28, 2004 Address correspondence to: David A. Whiting, 3600 Gaston Avenue, Suite 1058, Wadley Tower, Dallas, TX 75246, USA. whiting@ dallasassocderm.com Recommended Reading Benvenuto ME, Rebora A: Fluctuant nodules and alopecia of the scalp. Perifolliculitis capitis abscedens et suffodiens. Arch Dermatol 128: , 1992 Bergfeld WF, Elston DM: Cicatricial alopecia (and other causes of permanent alopecia). In: Olsen EA (ed). Disorders of Hair Growth: Diagnosis and Treatment, 2nd edition. New York: McGraw Hill, 2003; p Bergfield W, Mulinari-Brenner F, McCamon K, Embi C: The combined utilization of clinical and histological findings in the diagnosis of trichotillomania. J Cutan Pathol 29: , 2002 Headington JT: Transverse microscopic anatomy of the human scalp: A basis for a morphometric approach to disorders of the hair follicle. Arch Dermatol 120: , 1984 Headington JT: Telogen effluvium. New concepts and review. Arch Dermatol 129: , 1993 Kossard S, Lee MS, Wilkinson B: Postmenopausal frontal fibrosing alopecia: A frontal variant of lichen planopilaris. J Am Acad Dermatol 36:59 66, 1997 Powell JJ, Dawber RPR, Gatter K: Folliculitis decalvans including tufted folliculitis: Clinical, histological and therapeutic findings. Br J Dermatol 140: , 1999 Solomon AR: The transversely sectioned scalp biopsy specimen. The technique and algorithm for its use in the diagnosis of alopecia. Adv Dermatol 9: , 1994 Sperling L, Homoky C, Pratt L, Sau P: Acne keloidalis is a form of primary scarring alopecia. Arch Dermatol 136: , 2000 Sperling L, Sau P: The follicular degeneration syndrome in black patients: Hot comb alopecia revisited and revised. Arch Dermatol 128:68 74, 1992 Sperling L, Solomon A, Whiting D: A new look at scarring alopecia. Arch Dermatol 136: , 2000 Sperling LC: An Atlas of Hair Pathology with Clinical Correlations. New York: Parthenon Publishing, 2003 Sperling LC, Lupton GP: The histopathology of non-scarring alopecia. J Cutan Pathol 22:97 114, 1995 Whiting DA: Diagnostic and predictive value of horizontal sections of scalp biopsy specimens in male pattern androgenetic alopecia. J Am Acad Dermatol 28: , 1993 Whiting DA: Chronic telogen effluvium: Increased scalp shedding in middle-aged women. J Am Dermatol 35: , 1996 Whiting DA: Cicatricial alopecia: Clinico-pathological findings and treatment. Clin Dermatol 19: , 2001 Whiting DA: Histopathologic features of alopecia areata: A new look. Arch Dermatol 139: , 2003 Whiting DA: The Structure of the Human Hair Follicle: Light Microscopy of Vertical and Horizontal sections of Scalp Biopsies. Fairfield, NJ: Canfield Publishing, 2004