Pi Is 0190962208009328

S126 Letters

J AM ACAD DERMATOL NOVEMBER 2008

Scalp sarcoidosis with systemic involvement To the Editor: Cutaneous sarcoidosis is not uncommon, and the skin can be the first and only area affected.1-5 On the other hand, permanent alopecia caused by sarcoidosis is rare and is usually characterized by a few patches of cicatricial alopecia resembling discoid lupus erythematosus.1 We report a case of extensive cicatricial alopecia with systemic involvement caused by sarcoidosis in a 45-year-old Italian female. Alopecia involved the scalp almost entirely, extending from the frontal hairline to the vertex and both parietal areas. The scalp was diffusely shiny and atrophic, with areas of hyper- and hypopigmentation. No signs of active inflammation were present. A few tufted hairs were irregularly distributed on the frontal and occipital hairline. The patient revealed that she had been affected by hair loss for more than 15 years, but she had never previously received any specific treatment. Moreover, a few slightly atrophic, scaly red-brown papules and plaques had recently appeared on the forehead, temples, and ears, and this finally prompted her to seek medical advice. Her medical history revealed that the patient had been under treatment with systemic steroids for pulmonary sarcoidosis for 5 years. Her therapy at the time was deltacortene 5 mg every other day. A 4-mm punch biopsy and a 0.6 3 0.4 cm longitudinal biopsy from an involved area of the scalp, for vertical and horizontal sections, respectively, led to the diagnosis of sarcoidosis (Figs 1 and 2). Therapy with hydroxychloroquine (200 mg twice a day) and clobetasol ointment was started and the patient was followed every 6 weeks for 12 months. After 1 year, the alopecia was without signs of progression, and the facial lesions had considerably improved. The scalp is rarely affected in sarcoidosis and the diagnosis may be very difficult if other cutaneous or extracutaneous sites are not involved. Alopecia may start with an atrophic patch, sometimes with redness and scaling, usually within a localized area. It often presents as a scarring alopecia that clinically resembles discoid lupus erythematosus or lichen planopilaris. Reviewing the English language literature, fewer than 30 cases of scalp sarcoidosis have been reported, mostly involving African American female patients with systemic involvement.1,2 Our case is unusual because the patient had been suffering from hair loss for more than 10 years before the systemic disease and the cicatricial alopecia

Fig 1. Red scaly patches of the forehead, left temple, and auricle.

Fig 2. Horizontal sections. Nodular, granulomatous dermatitis. Collections of epithelioid histiocytes, some multinucleate, aligned in a palisade around foci of degenerated collagen and necrosis of inflammatory cells, surrounded by numerous lymphocytes at infundibular, isthmus level of hair follicle in the dermis. (Hematoxylin-eosin stain; original magnification: 310.)

involved most of the scalp with a clinical picture that closely resembled lichen planopilaris. A review of the literature also reveals that scalp sarcoidosis is quite often associated with systemic involvement.2 Despite limited clinical data, this indicates that a careful work-up is needed when scalp sarcoidosis is diagnosed. Our case demonstrates that a very long period of time may elapse between the cutaneous and extracutaneous symptoms. It is likely that a prompt initiation of therapy may have led to a different disease course. Histologically, tubercoloid granulomas, characterized by collections of epithelioid histiocytes surrounded by a moderately dense infı`ltrate of lymphocytes, may be seen in not only lupus vulgaris and tubercoloid leprosy, but also in sarcoidosis.6 Therapeutic options for scalp sarcoidosis are not satisfactory.1-3 Steroids, both systemic and intralesional, other immunosuppressive drugs, and

Letters S127

J AM ACAD DERMATOL VOLUME 59, NUMBER 5

antimalarials must be considered. We used hydroxychloroquine and obtained relatively good results in terms of remission of the facial lesions. Michelangelo La Placa, MD, Colombina Vincenzi, MD, Cosimo Misciali, MD, and Antonella Tosti, MD Department of Clinical and Experimental Medicine, Dermatology Section, University of Bologna, Bologna, Italy Funding sources: None. Conflicts of interest: None declared. Correspondence to: Michelangelo La Placa, MD, Department of Clinical and Experimental Medicine, Dermatology Section, University of Bologna, Via Massarenti 1, 40138 Bologna, Italy

Fig 1. Multiple discrete erythematous macules and papules, some with central vesicles, on the palmar creases and insteps.

E-mail: [email protected] REFERENCES 1. Henderson CL, Lafleur L, Sontheimer RD. Sarcoidal alopecia as a mimic of discoid lupus erythematosus. J Am Acad Dermatol 2008;59:143-5. 2. Katta R, Nelson B, Chen D, Roenigk H. Sarcoidosis of the scalp: a case series and review of the literature. J Am Acad Dermatol 2000;42:690-2. 3. Harman KE, Calonje E, Robson A, Black MM. Sarcoidosis presenting as a scarring alopecia resembling necrobiosis lipoidica. Clin Exp Dermatol 2003;28:565-6. 4. Akhdari N, Skalli HD, Lakhdar H. Erythematous lesions on the scalp. Sarcoidosis. Arch Dermatol 2004;140:1003-8. 5. Cho HR, Shah A, Hadi S. Systemic sarcoidosis presenting with alopecia of the scalp. Int J Dermatol 2004;43:520-2. 6. Ball NJ, Kho GT, Martinka M. The histologic spectrum of cutaneous sarcoidosis: a study of twenty-eight cases. J Cutan Pathol 2004;31:160-8. doi:10.1016/j.jaad.2008.07.041

Erythema multiformeelike presentation of chronic graft versus host disease To the Editor: A 53-year-old male with a history of multiple myeloma presented 6 months after an autologous stem cell transplant followed by an human leukocyte antigenematched allogeneic stem cell transplant with small, diffuse, erythematous confluent papules on his arms, legs, and trunk in addition to several oral mucosal erosions. The diagnosis of chronic graft versus host disease (GVHD) was made, and the patient was started on a slow prednisone taper, which led to complete resolution. One year later, he presented with a 4-month history of acute onset tender, erythematous papules and vesicles localized to the palmar creases and insteps. The lesions had remained unchanged in number and size

Fig 2. Biopsy specimen from a representative papule shows vacuolization of the basal layer, scant papillary dermal lymphocytic infiltrate, and individual necrotic keratinocytes with apposition of lymphocytes. (Hematoxylineeosin stain; original magnification: 340.)

but were becoming increasingly painful. Physical examination revealed numerous 2- to 6-mm discrete erythematous macules and papules, many of which had a central vesicle and variable scale. They were confined to the palmar creases and insteps, with only a few random vesicles present on the fingertips. Many appeared targetoid because of an erythematous halo surrounding a central vesicle (Fig 1). Inspection of the mouth revealed several palatal erosions. Punch biopsy specimens were obtained from the patient’s right hand and left foot. Histopathologic analysis revealed individual necrotic keratinocytes with apposition of lymphocytes (‘‘satellite cell necrosis’’) in the basal and mid spinous layers of the epidermis and basal cell vacuolization and a scant papillary dermal lymphocytic infiltrate (Fig 2). A diagnosis of chronic GVHD was made, and the patient was started on a slow prednisone taper and tacrolimus ointment with some improvement. Later, narrow band ultraviolet B light phototherapy followed by 3 months of extracorporeal photophoresis