A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. These patients showed generalized follicular hyperkeratosis and diffuse thickening of the palms and soles. Histopathological findings consisted of keratotic plugging of the follicular infundibulum and features of erector pili myositis. We report on an 18-year-old woman with dermatomyositis. The diagnosis was established by characteristic enzymatic alterations, electromyographic pattern of myositis and the findings in a muscle biopsy, although the patient had no evidence of muscular weakness during a follow-up of 14 years. She developed an erythematosus and squamous eruption associated with diffuse palmoplantar keratoderma. Histopathological features consisted of a papillomatous epidermis with spicules of compact eosinophilic hyperkeratosis over the tips of papillae that were not related to hair follicles. Pityriasis rubra pilaris-like eruption seems to be a characteristic although uncommon cutaneous manifestation in dermatomyositis.

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. These patients showed generalized follicular hyperkeratosis and diffuse thickening of the palms and soles. Histopathological findings consisted of keratotic plugging of the follicular infundibulum and features of erector pili myositis. We report on an 18-year-old woman with dermatomyositis. The diagnosis was established by characteristic enzymatic alterations, electromyographic pattern of myositis and the findings in a muscle biopsy, although the patient had no evidence of muscular weakness during a follow-up of 14 years. She developed an erythematosus and squamous eruption associated with diffuse palmoplantar keratoderma. Histopathological features consisted of a papillomatous epidermis with spicules of compact eosinophilic hyperkeratosis over the tips of papillae that were not related to hair follicles. Pityriasis rubra pilaris-like eruption seems to be a characteristic although uncommon cutaneous manifestation in dermatomyositis.

A 37‐year‐old white man with a history of essential arterial hypertension and idiopathic thrombocytopenic purpura (for the latter disorder, oral corticosteroids had been administered successfully), was first examined in our department in June 1997. He had a persistent, pruriginous eruption on the pretibial regions which had been present since the age of 16 years. He complained of bullous lesions after minor trauma to the skin. Examination revealed bilateral, brown, erythematous, shiny, indurated plaques on the anterior regions of his lower legs. Several clear blisters, scars, and excoriations were also present on the left leg ( ). His toenails were thickened and dystrophic, but his teeth and hair were normal. 1 Vesicles, erosions, and crusts on the left leg Histopathologic examination of a vesicle on the left leg revealed a subepidermal blister with sparse perivascular lymphocytic infiltrate in the upper dermis. The contents of the blister were fibrinous ( ). Immunohistochemical study was automatically performed (TechMate 500) with monoclonal antibody to type IV collagen (clone CIU 22, Dako), a component of lamina densa, after enzymatic and heat antigen retrieval. Type IV collagen was present on the roof of the blister, indicating that the blister formation was beneath the lamina densa ( ). Ultrastructural examination showed an intact dermoepidermal junction with normal hemidesmosomes and basal keratinocytes; however, the anchoring fibrils were markedly decreased in number ( ) and were rudimentary. 2 (A) A subepidermal blister covered by intact epidermis and sparse inflammatory infiltrate in the papillary dermis (hematoxylin and eosin, ×100). (B) Immunohistochemical investigation for collagen IV demonstrated that the blister developed beneath the lamina densa (collagen IV, ×200) 3 Ultrastructural examination showed a decreased number of anchoring fibrils in the dermal papillae (×12,000) The father of the patient and the paternal grandfather had a similar blistering eruption on the pretibial areas. His son, a 2‐year‐old boy, also presented bullous lesions on the anterior surface of the legs, with scars and milia. His daughter, a 6‐year‐old girl, was not affected. The pattern of inheritance was autosomal dominant.

Few cases of naevoid hyperkeratosis of the nipple and areola (NHNA) have been documented since the condition was first described in 1923. A recent PubMed search (Mar 2014) yielded 107 cases of NHNA in the world literature, mostly female patients with adolescent-onset disease. We report the first two cases of adolescent-onset NHNA from the Philippines. To our knowledge, these are also the first two cases of extensive bilateral NHNA reported in which the lesions have extended beyond the areolae affecting the surrounding periareolar skin, covering almost the entire breast. Medical treatment of NHNA has been commonly associated with treatment failure or recurrence after treatment cessation, requiring intermittent therapy. However, our two cases had successful outcomes with medical treatment using a combination topical steroid/salicylic acid ointment with > 2 years of follow-up.