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Two siblings had hyperpigmented indurations over the inner aspects of both thighs extending to the lower abdomen. Skin biopsy showed plasma cell panniculitis favoring a diagnosis of morphea profundus. Family history of consanguinity was present, but both parents were unaffected. To our knowledge this is the first report of autosomal recessive plasma cell panniculitis with the clinical manifestations of morphea.

Background: Linear IgA bullous dermatosis of childhood is a rare autoimmune bullous disease that mainly affects preschool-aged children. Dapsone is considered the first-line therapy with prompt response from most patients. However, it may be contraindicated in certain conditions such as glucose-6-phosphate dehydrogenase deficiency., Objective: We sought to assess the efficacy of flucloxacillin in the treatment of linear IgA bullous dermatosis., Methods: This is an observational study in which all confirmed cases of linear IgA bullous dermatosis (by both histological and immunofluorescence studies) will be treated with flucloxacillin. Flucloxacillin will be continued according to the response or otherwise will be discontinued after 8 weeks in the case of resistance., Results: We describe 7 patients with linear IgA bullous dermatosis of childhood treated with flucloxacillin. In 4 cases, it induced complete remission within 3 to 4 months of starting therapy with no relapses. In the other 3 cases, it successfully controlled the disease but with prompt relapse on discontinuation of the treatment., Limitations: This is a case series study with a small number of patients., Conclusion: Flucloxacillin may be considered among the first alternative therapies for linear IgA bullous dermatosis of childhood. Further evaluation of the efficacy and safety of the long-term use is required.

We report a 19-year-old man with congenital erythematous plaque on his left arm that was found histopathologically to be composed of respiratory mucosa. The patient had a triphalangeal thumb and polydactyly in the left hand. This is, to our knowledge, only the third case of ectopic respiratory epithelium presenting as a superficial lesion in the skin to be reported in the English literature, and the first case associated with skeletal malformation and polydactyly.