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A 46-year-old woman was diagnosed with Brooke-Spiegler syndrome (BSS) with lesions on her head, predominantly on the center of her face, auricles, and scalp. The facial lesions were polymorphic, pale, confluent, skin-colored, and papular, whereas the scalpel lesions appeared as erythematous and violet lumps that varied in size (Figure 1A, B).

Actinic prurigo is an idiopathic photodermatosis that affects the skin, as well as the labial and conjunctival mucosa in indigenous and mestizo populations of Latin America. It starts predominantly in childhood, has a chronic course, and is exacerbated with solar exposure. Little is known of its pathophysiology, including the known mechanisms of the participation of HLA-DR4 and an abnormal immunologic response with increase of T CD4+ lymphocytes. The presence of IgE, eosinophils, and mast cells suggests that it is a hypersensitivity reaction (likely type IVa or b). The diagnosis is clinical, and the presence of lymphoid follicles in the mucosal histopathologic study of mucosa is pathognomonic. The best available treatment to date is thalidomide, despite its secondary effects.

A 50-year-old-woman presented with a history of crusted plaques on her face and trunk. On physical examination, she had a disseminated dermatosis involving the face, chest, trunk, abdomen, and both ankles. She had erythematous and scaly plaques on both cheeks (Figure 1). In both ears and on the rest of the body, she presented with scarce intact vesicles and blisters, a few eroded and crusted vesicles, and residual hyperpigmented macules. The onset of the dermatosis occurred 6 months before diagnosis, affecting the chest, followed by further spreading of vesicles on the body. She denied ever having developed ulcerative or erosive lesions on mucosal surfaces. The presence of blisters were followed by the development of crusts and hyperpigmented macules. One month before visiting our service, the patient had been receiving treatment with dapsone, with slight improvement of the cutaneous lesions. Biopsy results of one of the scaly plaques on the cheeks demonstrated a subcorneal blister with a few acantholytic cells and a scarce inflammatory infiltrate in the papillary dermis (Figure 2). Because of the clinical picture, especially the erythematous lesions on both cheeks, tests for antinuclear antibodies were performed to rule out pemphigus erythematosus, and the results were negative. Besides the cutaneous manifestations, the patient had had signs and symptoms of arthritis for at least 11 years; she had received sporadic relief with the nonsteroidal anti-inflammatory drug ketoprofen. She reported generalized morning rigidity lasting for 2 hours and arthralgias in the temporomandibular joints, elbows, wrists, metacarpophalangeal joints, knees, and ankles. On examination, she had phlogosis and synovial thickening in the elbows, wrists, metacarpophalangeal joints, knees, and ankles. She also had flexion contractures on both elbows as well as ulnar deviation of the left hand and swan-neck deformity in several fingers. Radiographic examination of the hands demonstrated generalized osteopenia, decreased articular spaces in metacarpophalangeal and proximal interphalangeal joints, and slight erosion in the carpal bones (Figure 3). Results of rheumatoid factor testing were positive (521 IU/mL). The diagnoses of both pemphigus foliaceus and rheumatoid arthritis were made. We performed high-resolution DNA typing of HLA-DRB1 polymorphism using sequence-specific oligonucleotides,1 and found that the patient carried HLA DRB1*0404 and *1406. Figure 1. The patient had erythematous and scaly plaques on both cheeks. Figure 2. Biopsy revealed a subcorneal blister with a few acantholytic cells and a scarce inflammatory infiltrate in the papillary dermis. Figure 3. Radiography of the hands, revealed generalized osteopenia, decreased articular spaces in metacarpophalangeal and proximal interphalangeal joints, and slight erosion in the carpal bones.