Dowling-Degos disease (DDD) is an autosomal dominant genodermatosis and this disease is a genetically determined disturbance of epidermal proliferation. It is characterized by acquired, slowly progressive pigmented lesions that primarily involve the great skin folds and flexural areas such as the axilla, neck, limb flexures, the inframammary area and the inguinal folds. The vulva is an unusual location for DDD. A 41-year-old woman presented with a 10-year history of multiple, small, reticulated and brownish macules distributed symmetrically on the bilateral external genital regions. We found no other similarly pigmented skin lesions on her body, including the flexural areas. There was no known family history of similar eruptions or pigmentary changes. The histologic examination showed irregular rete ridge elongation with a filiform or antler-like pattern and basilar hyperpigmentation on the tips. Fontana-Masson staining showed increased pigmentation of the rete ridges and the S100 protein staining did not reveal an increased number of melanocytes in the epidermis. From these findings, we diagnosed this lesion as DDD.
Hereditary hemorrhagic telangiectasia, also known as Osler-Weber-Rendu disease, is an autosomal dominant disorder of the fibrovascular tissue. It is characterized by the classic triad of mucocutaneous telangiectasias, recurrent hemorrhages, and familial occurrence. The cutaneous manifestation appear clinically as punctuate, linear, or splinter-like telangiectasias of the upper body, oral, and nasal mucous membranes, and nail beds. A 73-year-old woman presented with purpuric, punctuate, and tiny macules on the finger tips of both hands and the tongue. The skin lesions were discovered about 50 years previously. She had a family history of cutaneous telangiectasia. Also, she had episodes of recurrent epistaxis, gastrointestinal bleeding, and anemia. The gastroendoscopy revealed gastric angiodysplasia of the fundus and body of the stomach. The histopathologic study showed dilated capillaries lined by flat endothelial cells in the papillary dermis. From these findings, we diagnosed this case as hereditary hemorrhagic telangiectasia, which has rarely been reported in the dermatologic literature.
Department of Dermatology, Guri Hospital, Hanyang University College of Medicine, Guri, KoreaA digital mucous cyst (DMC) is clinically characterized by a round to oval, translucent, smooth nodule localized to the dorsal aspect of the distal digits near the distal interphalangeal joint. It usually presents as a solitary lesion, and multiple lesions are uncommon. An 88-year-old man presented with herpetiform translucent papules on the right thumb. We first diagnosed the lesion as molluscum contagiosum or herpetic whitlow. Histopathology showed a cystic space containing mucinous material and numerous fibroblasts surrounded by mucinous stroma in the upper dermis. The lining of the cyst wall was not apparent and mucinous material was stained with Alcian blue, indicating a diagnosis of DMC. (Ann Dermatol 22(2) 194∼195, 2010)
Lichen planus (LP) commonly involves the flexor aspects of the wrists, legs, and oral and genital mucous membranes. But it rarely occurs on the palms and/or soles. It mainly affects people in the age range 30∼60 years. Childhood LP is reported to constitute only 1∼4% of total cases of LP. In the literature, a few cases of LP on the palms and soles of pediatric patients have been reported. Here we report an interesting case of childhood LP with palmoplantar involvement. The patient was a 7-year-old boy who for 6 months had variously sized and shaped, pruritic, violaceous, polygonal papules and plaques on the whole body. The skin biopsy specimens taken from four sites of the patient (dorsum of the hand and foot, sole and chest) showed typical features of LP on histopathology. A diagnosis of generalized LP with palmoplantar involvement was made.
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