Guttate leukoderma and acrokeratosis verruciformis of Hopf: a rare combination in Darier disease.

A distinct Darier phenotype presenting with confetti-like hypopigmented macules was first described in 1965. Designated as "guttate leukoderma," this skin finding is a rarely-reported presentation of Darier disease. It has been theorized that the mutation in ATP2A2 causes defective E-cadherin, which in turn disrupts the adhesion of melanocytes to keratinocytes, thus leading to impaired dendrite formation, hindered melanin transfer, and ultimately to melanocyte apoptosis. Herein, we contribute a case of a 56-year old woman who presented with the rarely-described guttate leukoderma of Darier disease and acrokeratosis verruciformis of Hopf.

Erythropoietic Protoporphyria: You May Not Have Seen It, but It May Have Seen You.

Erythropoietic protoporphyria is a rare skin condition that commonly presents in childhood. We report a case of a 35-year-old Hispanic male with a history of sun sensitivity, presenting with complaints of immediate burning and itching of the skin on his face and upper extremities upon sun exposure. On examination, there was minimal face erythema and calluses over the knuckles. Laboratory workup demonstrated substantially increased protoporphyrin (over 10 times the upper limit of normal) along with elevated liver enzyme levels. Liver biopsy confirmed stage 4 cirrhosis. Our patient's cutaneous manifestations were the primary complaint that led to the diagnosis of his terminal hepatic illness. We recommend screening for erythropoietic protoporphyria in patients who present with a life-long history of non-blistering, burning and itching of the skin, which begins immediately upon sun exposure.

Central Centrifugal Cicatricial Alopecia and Lichen Planopilaris Can Look Identical on Histopathology.

Central centrifugal cicatricial alopecia (CCCA) and lichen planopilaris (LPP) can look identical on histopathology. Both entities demonstrate perifollicular fibrosis, peri- infundibular lymphocytic infiltrates, and reduced follicular density. Presence of normal follicular units and preservation of sebaceous glands favor CCCA. An elastin stain, that is, Verhoeff-Van Gieson stain, can differentiate the tree trunk fibrosis of CCCA from the narrow wedge-shaped fibrosis of LPP. Look for important clues such as patient race and pattern of alopecia for diagnosis and treatment instead of relying solely on the pathology report.