Volume 27 Number 2, Part 2 August 1992

gressive basal cell carcinomas that begin at an early age, along with jaw cysts, skeletal anomalies, ectopic calcification, and pits of the palms and soles. The other cutaneous adnexal tumors can be easily differentiated through histopathologic methods. Most reported cases of linear BFH do not mention the treatment and follow-up of the patients. In one case the BFH was entirely excised and replaced with a full-thickness skin graft. 6 Because of the possible development of basal cell carcinomas within the BFH, the patient should be observed periodically and small basal cell carcinomas should be excised as soon as they appear. We thank Orlando Sufirez and Dr. A. Rey Lrpez for their technical assistance with the photographic material. REFERENCES 1. Brown AC, Crounse RG, WinkelmannRK. Generalized h air follicle hamartoma associated with alopecia, aminoaciduria and myasthenia gravis. Arch Dermatol 1969;99:478-93.

Basaloid hamartoma 2. Ridley CM, Smith N. Generafized hair follicle hamartoma associated with alopeeia and myasthenia gravis: report of a second case. Clin Exp Dermatol 1981;6:283-9. 3. Weltfriend S, David M, Ginzburg A, et al. Generalized hair follicle hamartoma: the third case report in association with myasthenia gravis. Am J Dermatopathol 1987;9:428-32. 4. Carney RG. Linear unilateral basal cell nevus with comedones. Arch Dermatol Syph 1952',65:471-6. 5. Bleiberg J, Brodkin RH. Linear unilateral basal cell nevus with comedones. Arch Dermatol 1969;100:I87-90. 6. Anderson TE, Best PV. Linear basal cell naevus. Br J Dermatol 1962;74:20-3. 7. Mehregan AH, Baker S. Basaloid follicular hamartoma: three eases with localized and systematized unilaterallesions. J Cutan Pathol 1985;12:55-65. 8. Geffner RE, Goslen JB, Santa Cruz D J. Linear and dermatomal trichoepitheliomas. J AM ACAD DERMATOL1986; 14:927-30. 9. Gutierrez MM, Morn RG. Nevoid basal cell carcinoma syndrome: a review and case report of a patient with unilateral basal cell nevus syndrome. J AM ACAD DERMATOL 1986;15:1023-30.

Generalized perforating granuloma annulare Curt P. Samlaska, MAJ, MC, FACP, a Glenn D. Sandberg, CPT, MC, b Kurt L. Maggio, CPT, MC, c and E. Lawrence Sakas, LTC, MC, F C A P b Honolulu, Hawaii Generalized perforating granuloma annulare is characterized by 1 to 4 mm umbilicated papules on the extremities, and is most commonly seen in children and young adults. Transepithelial elimination of mucinous, degenerating collagen fibers and surrounding palisading lymphohistiocyticgranulomas are important histologic features. Perforating sarcoidosis and perforating granuloma annulare may be difficult to differentiate because of a similar clinical appearance and the presence of sarcoidal granulomas in biopsy specimens. A particularly high incidence of perforating granuloma annulare has been reported in the Hawaiian Islands. (J AM ACAD DERMATOL1992;27:319-22.) Although Fox I initially described the features of classic granuloma annulare in 1895, it was not until 1902 that Radcliffe-Crocker 2 labeled the disorder as a specific entity.l-3 Classic features include single or multiple flesh-colored papules and expanding annuFrom the Dermatology Service,a Department of Pathology,b and Department of Medicine,c Tripler Army Medical Center. The opinionsor assertionscontainedhereinare the privateviewsof the authorsand are not to be construedas officialor as reflectingthe views of the Departmentof the Army or the Departmentof Defense. Reprints not available. 16/4/36890

lar plaques that are comprised of small papules on the extremities. Although granuloma annulare is primarily a disease of children and young adults, it has been reported in virtually all age groups. 4 Females are affected twice as often as males, and familial occurrence is rare) N o associated systemic sequelae are present, and laboratory evaluation is usually normal. Pathognomonic features include granulomas that are composed of degenerating mucinous collagen, which is surrounded by palisading lymphoid cells and histiocytes. 3,6 This characteristic histologic appearance has resulted in the iden319

Journal of the American Academy of Dermatology

Samlaska et aL

320

L,'

, l"

"

l'

'

,,

'

L

,'

'

,,', '

,

?'

,

,~

'

,',II

"

'

,'

,' '

"~,.:'...!"~. ::.;;',."' '.'

' '

',

'f"

;"'

'~ ,

"'~'"

.........

,'

', ,,

,

"'',

":'

, '

,

' ,"

,",'

,

, '

'"

,,'', '

'

'

'

",'"'

l

',"'~','i

.''. " "...'........ ;."~'L~'I .... " ;' ~'i':ii''' "..... :'~J~!~i'"~::i.q~,;.~l

I .', II

i

The lesions appeared abruptly 2 years previously, shortly after the patient had moved to Hawaii. The patient was unaware of an association with sun exposure. The family history included no cutaneous diseases or diabetes. A chest roentgenogram and screening serum electrolytes were normal. The pretibial and ankle areas were most significantly involved; only a few papules were noted on the upper extremities. Clinical evaluation revealed hundreds of 1 to 2 mm umbilicated papules with central scaling (Fig. 1). Shave and punch biopsies of the dorsum of the right foot were performed. Because of a lack of symptoms, the patient refused treatment.

Case 2 A 5-year-old Oriental girl had numerous 1 to 3 ram, mildly pruritic, umbilicated papules on the arms and legs (Fig. 2). The lesions were particularly prominant on the distal extremities. A punch biopsy specimen was obtained from the right hand. Treatment with topical steroid preparations controlled the pruritus, but the cutaneous lesions remain 2 years after onset.

Case 3

Fig. 1. Multiple umbilicated papules that involve distal extremities in a 24-year-old woman. Fig. 2. Severe involvement of upper and lower extremities in a 5-year-old child.

tification of a variety of clinical forms: localized, generalized, macular, erythematous, subcutaneous nodular, and perforating granuloma annulare. 6, 7 Generalized granuloma annulare is classified separately from the localized form because of the possible association with diabetes, a later age of onset (it predominantly occurs in middle-aged women), rare spontaneous resolution, and poor response to therapy.7, s The differential diagnosis of classic granuloma annulare includes sarcoidosis, lichen planus, urticaria pigmentosa, necrobiosis lipoidica diabeticorum, and rheumatoid nodules. 6, 7 Generalized perforating granuloma annulare is a rarely reported form that has a high prevalence in the Hawaiian Islands. The three cases reported herein were seen during the past year. CASE REPORTS Case 1 A 24-year-old white woman had asymptomatic scaling papules that were distributed over the distal extremities.

An 1 l-year-old white girl had numerous pruritic, umbilicated papules on the left wrist and hand. The eruption began at the age of 5 years with lesions on the arms, elbows, and both legs. At the time of onset she lived in Florida. Treatment with intralesional and topical steroids resulted in some improvement, but not complete resolution. At the time of examination the only sites involved were the left wrist and hand. Clinical examination revealed numerous 1 to 3 mm umbilicated papules with surrounding lichenification and postinflammatory changes (Fig. 3). The patient was treated with clobetasol propionate with complete resolution within 1 month of treatment.

Histopathology Each case demonstrated mucinous collagen degeneration with transepithelial elimination (Fig. 4). The mucinous collagen was surrounded by palisading granulomas that were composed of lymphoid cells and histiocytes. In Case 1, sarcoidal granulomas were found along the dermoepidermal junction. Degenerating collagen showed metachromatic staining with alcian blue. DISCUSSION Calnan 9 described the clinical and histologic features of this disorder in 1954, and Owens and Freeman 1~ introduced the term perforating granuloma annulare in 1971. To the best of our knowledge, only 29 cases have been documented in the literature, including the cases reported herein.4" 11-14izumil5 was the first to describe this disorder in the Hawaiian Islands, and subsequently has collected some 20 cases

Volume 27 Number 2, Part 2 August 1992

(personal communication). Although perforating granuloma annulare is reported to be a rare disorder, it is not uncommon in the Hawaiian Islands, and members of the Hawaii Dermatologic Society observe one to three cases in their clinical practices each year (personal communication). Perforating granuloma annulare occurs most frequently in childhood, and it appears as 1 to 4 mm umbilicated papules that involve the extremities.< 10, 11-17In some patients the following progressive stages may be observed: (l) erythematous papules, which evolve into (2) yellowish, pustularappearing lesions, which subsequently discharge (3) a clear fluid. < 11 The appearance of larger, more ulcerated plaques (1 to 4 cm in size), as seen in generalized forms of granuloma annulare, is most common in middle-aged and elderly patients and may be associated with diabetes, i i An asymptomatic course with spontaneous resolution is the usual outcome. In Case 1 results of a shave biopsy specimen of a group of umbilicated papules resulted in the identification of sarcoidal granulomas and transepithelial elhnination of collagen. "Naked" granulomas were observed at the dermoepidermal junction, which suggests the possibility of sarcoidosis. Previous case reports used punch biopsy specimens, which help identify deeper granulomatous involvement and transepithelial elimination in mature lesions. Results of a shave biopsy specimen in our case resulted in a sampling of multiple lesions that were in various stages of development. For this reason "sarcoidal" granulomas at the dermoepidermal junction may be more commonly associated with perforating granuloma annulare than previously reported, because of selective sampling of more mature lesions. The differential diagnosis of perforating granuloma annulare includes molluscum contagiosum, perforating c.ollagenosis, elastosis perforans serpiginosa, perforating folliculitis, and sarcoidosis. It can be difficult to distinguish perforating granuloma annulare from sarcoidosis on histologic and clinical grounds. This emphasizes the need for thorough sectioning of biopsy tissue to identify transepithelial elimination of mucinous degenerating collagen in those cases in which "sarcoidal" granulomas are identified. Many authors, however, believe that perforating granuloma annulare has a distinct clinical appearance. 15-17 Three cases of sarcoidosis with transepithelial elimination have been reported. 18-20Cutaneous involvement occurred in the head and neck regions, with atrophic annular plaques or small papular

Generalizedperforating granuloma annulare 321

1

Fig. 3. Eleven-year-old child with a 6-year history of lesions that involve left wrist and hand. Fig. 4. Results of a punch biopsy specimen demonstrate transepithelial elimination of degenerating collagen and palisading granulomas. (Hematoxylin-eosin stain; • lesions in all three cases. The case reported by Batres et al. ~9 also featured scrotal involvement. The case reported by Balus and Bassetti 2~ had numerous lesions on the lower extremities, which provided a clinical picture that is similar to perforating granuloma annulare. The cause of granuloma annulare is unknown. Ultraviolet light, insect bites, trauma, viral infections, thyroiditis, and vitamin D have been implicated, but are unproved etiologic factors, lq7 Although the large number of cases seen in the Hawaiian Islands suggests an association with ultraviolet light exposure, attempts to reproduce these lesions have been unsuccessful. Is Seasonal varia-

322

Samlaska et al.

tions h a v e been described, with clearing of lesions during t h e winter and exacerbations during the summer.16 An association between insect bites (such as Culicoides furens) and perforating granuloma annulare has been suspected, but is unproven. 15 Perhaps some unidentified environmental factors play a significant role in the cause of this disorder. Severe variants of g r a n u l o m a annulare, particularly the generalized form, m a y necessitate therapeutic intervention. Intralesional steroid injections are the t r e a t m e n t of choice for m o r e localized disease. 7 O t h e r forms o f t r e a t m e n t that have been reported to have variable success are topical steroids, dapsone, 21 retinoids, 7 niacinamide, 22 potassium iodide,23 chloroquine, c r y o t h e r a p y , surgery, electrodessication, a n d x-ray therapy. MT' 21-22 REFERENCES 1. Fox TC. Ringed eruption of the fingers. Br J Dermatol 1895;7:91-2. 2. Radcliffe-Crocker H. Granuloma annulare. Br J Dermatol 1902;14:1-9. 3. Moyer DG, Heights B. Papular granuloma annulare. Arch Dermatol 1964;89:41-5. 4. Shelnitz LB, Esterly NB. Umbilicated papular eruption on the extremities of a child. Arch Dermatol 1986;122:93l-6. 5. Friedman SJ, Winkelmann RK. Familial granuloma annulare. J AM ACADDERMATOL1987;16:600-5. 6. Lever WF, Scha~tmhurg-Lever G. Noninfectious granulomas. In: Lever WF, Schaumburg-Lever G, eds. Histopathology of the skin. 7th ed. Philadelphis: JB Lippincott, 1990:257-60. 7. Arnold HL, Odom RB, James WD. Erythema and urticaria. In: Arnold HL, Odom RB, James WD, eds. Andrew's diseases of the skin. 8th ed. Philadelphia: WB Saunders, 1990:144-7.

Journal of the American Academy of Dermatology

8. Dabski K, Winkelmann RK. Generalized granuloma annulare: clinical and laboratory findings in 100 patients. J AM ACADDERMATOL1989;20:39-47. 9. Cainan CD. Granuloma annulare. Br J Dermatol 1954; 66:254. 10. Owens DW, Freeman RG. Perforating granuloma annulate. Arch Dermatol 1971;103:64-7. 11. Shimizu H, Harada T, Kuramoehi M. Perforating granuloma annulare. Int J Dermatol 1985;24:58l-3. 12. Huerter C J, Bass J, Bergfeld WF, et al. Perforating granuloma annulare in a patient with acquired immunodeficiency syndrome. Arch Dermatol 1987;123:1217-20. 13. Gutierrez MC, Hasson A, Arias D, et al. Perforating annular granuloma in an atypical location. Dermatol Ibero Lat Am 1990;18:5-8. 14. Wright AL, Buxton PK, Mclaren KM. Perforating granuloma annulare. Int J Dermatol 1989;28:466-7. 15. Izumi AK. Generalized perforating granuloma annulare. Arch Dermatol 1973;108:708-9. 16. Duncan WC, Smith JD, Knox JM. Generalized perforating granuloma annulare. Arch Dermatol 1973;108:570-2. 17. Delaney T J, Gold SC, Leppard B. Disseminated perforating granuloma annulare. Br J Dermatol 1973;89:523-6. 18. Goette DK, Odom RB. Transepithelial elimination of granulomas in cutaneous tuberculosis and sarcoidosis.J AM ACAD DERMATOL1986;14:126-8. 19. Batres E, Klima M, Tschen J. Transepithelial elimination in cutaneous sareoidosis. J Cutan Pathol 1982;9:50-4. 20. Balus L, Basetti G. Sarcoidosis with transepithelial elimination. Chronica Dermatol 1979;10:67-74. 21. Steiner A, Pehamberger H, Wolff K. Sulfone treatment of granuloma annulare. J AM ACAD DERMATOL 1985;13: 1004-8. 22. Ma A, Medeniea M. Response of generalized granuloma annulare to high-dose niacinamide. Arch Dermatol 1983; 119:836-9. 23. Caserio R J, Eaglstein WH. Treatment of granuloma annulare with potassium iodide [Letter]. J AM ACADDERMATOL1984;10:294-5.

Generalized perforating granuloma annulare.

Generalized perforating granuloma annulare is characterized by 1 to 4 mm umbilicated papules on the extremities, and is most commonly seen in children...
2MB Sizes 0 Downloads 0 Views