Joung Soo Kim

A case of primary anetoderma in an infant

SIR, Anetoderma is a rare cutaneous disorder characterized by a localized depression or outpouching of the skin caused by laxity and weakening of the dermal connective tissue as a result of focal loss of elastic fibres. It is sometimes not associated with any underlying disease [primary (idiopathic) anetoderma] or it can be related to many kinds of dermatoses (secondary anetoderma). In the past, cases of primary anetoderma were classified into the Jadassohn–Pelizzari type, following erythema or urticaria, and the Schweninger–Buzzi type with no preceding inflammatory skin lesions. Currently, this classification is becoming less important because, whether or not one can recognize this feature, it is only an individual variable, not a disease-defining feature. Anetoderma occurs mainly in middle-aged women, often in children and rarely in infants. We report an interesting case of primary anetoderma, the Schweninger–Buzzi type, which occurred in a 1-month-old infant. To our knowledge, this is the youngest reported age of onset of a case that is not congenital. A 5-month-old boy presented with increasing numbers of multiple atrophic and sac-like whitish papules on his trunk and both extremities, which started to develop only 1 month after birth (Fig. 1a,b). He had no history of preceding inflammatory skin diseases and did not show any symptom associated with the development of the skin lesions. He was born after a full-term single pregnancy and had no problems during the perinatal period. The family history was not contributory. On clinical examination, the otherwise healthy-looking boy presented with 40–50 atrophic and sac-like round to ovalshaped whitish papules on the trunk, buttocks and both extremities. The lesions were 2–20 mm in diameter and were well circumscribed, presenting either as a depression below the level of normal skin or as a sac-like protrusion. Routine laboratory examinations were within normal limits. There were no neurological, orthopaedic or ophthalmological abnormalities. A biopsy was performed from a typical looking lesion on his back. Histopathological examination revealed a mild perivascular infiltrate with lymphocytes in the mid-dermis (Fig. 2a). The elastic stain demonstrated a decreased number of elastic fibres in the superficial and the mid-dermis (Fig. 2b). A 12-month period of treatment with clobetasone butyrate (0Æ5 mg g) cream showed no effective response and, during that time, we noticed about 10–15 newly developed skin lesions. We are still observing the patient every 2–3 months. Anetoderma, which was first described by Jadassohn in 1892, is characterized clinically by small, atrophic papules with flaccid skin, inward herniation at palpation and histopathological loss of elastic tissue. This rare disorder occurs

Cure rate, duration required for complete cure and recurrence rate of onychomycosis according to clinical factors in Korean patients

Many factors affect the cure rate (CR), duration required for complete cure (DC) and the recurrence rate (RR) of onychomycosis. The aim of this study was to evaluate the CR, DC and RR in onychomycosis according to various clinical factors. We retrospectively reviewed medical records of 637 Korean patients with onychomycosis between December 2000 and December 2006. We examined six clinical factors to evaluate the effects on the CR, DC and RR: age, sex, clinical type, treatment pattern, presence of diabetes mellitus (DM) and the extent of nail involvement. On the view of the clinical nail appearance and potassium hydroxide (KOH) preparation, we designated the CR, DC and RR. In addition, we examined the differences in the CR, DC and RR in terms of the above‐mentioned clinical factors. A total of 207 eligible patients were finally analysed. The CR as a whole was 78.3%, the DC was 31.7 ± 18.4 weeks and the RR was 36.0%. There were significant differences in the CR, DC and RR according to the extent of nail involvement. Age affects the CR and DC, and DM also affects the DC and RR. We found that the extent of nail involvement, age and DM affect the CR, DC and RR of onychomycosis.

Distinct variant of Sweet’s syndrome: bortezomib-induced histiocytoid Sweet’s syndrome in a patient with multiple myeloma

Histiocytoid Sweet’s syndrome (SS) is a rare variant of the acute febrile neutrophilic dermatosis recently described. It is characterized by an infiltrate of immature myeloid cells. Only one case of histiocytoid SS induced by bortezomib in multiple myeloma has been reported in the dermatologic literature. Herein, we present a case of bortezomib-induced histiocytoid SS in a patient with multiple myeloma following a course of bortezomib, along with a review of the literature.

A case of hereditary hemorrhagic telangiectasia.

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.

A Case of Pigmented Bowen's Disease

Pigmented Bowens disease is characterized by increased melanin pigment in the epidermis or papillary dermis, in addition to the typical findings of Bowens disease. This disorder has been infrequently reported and it represents less than 2% of all cases of Bowens disease. Thus far, there has been only 1 case of pigmented Bowens disease on the umbilicus in the medical literature, and no such case has been reported in Korea. Pigmented Bowens disease develops on sun-exposed areas of the face and neck, as well as sun-unexposed areas like the trunk, extremities, perianal area and umbilcus. A 36-year-old man presented with a 9-month history of solitary dark brown slithery or wavy plaque with a verrucous surface on the umbilicus, and the lesion measured 1 x 2 cm in size. The histopathologic findings showed hyperkeratosis, parakeratosis and atypical keratinocytes disorderly arranged throughout the epidermis. Increased melanin pigment was noted in the basal layer of the epidermis. From these findings, we diagnosed this lesion as pigmented Bowens disease.

Comparison of cure rates and durations of treatment with diphenylcyclopropenone immunotherapy for warts in children/adolescents and adults

Immunotherapy has been used for many years in the treatment of warts. Diphenylcyclopropenone is frequently used as an immunotherapeutic agent.

Merkel Cell Carcinoma Concurrent with Bowen's Disease

Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous malignancy of the elderly and immunocompromised patients. It is occasionally found coexisting with other diseases, such as squamous cell carcinoma, basal cell carcinoma, actinic keratosis, miscellaneous adnexal tumors, and rarely Bowen disease. A 75-year-old woman presented with a 6-month history of an irregularly shaped erythematous patch on the left mandibular angle. Three months later, a 1.5×1.0 cm sized painless and rapidly growing erythematous nodule developed on the patch. Microscopically, the patch lesion was consistent with that of Bowen disease. The nodular lesion showed a number of small uniform hyperchromatic cells with scanty cytoplasm. It showed dense small-cell like nodular infiltration in the dermis. Immunohistochemical staining for cytokeratin 20 showed a positive result with a dot-like perinuclear pattern. Additionally, the result for thyroid transcription factor-1 was negative, which is positive in small cell neuroendocrine carcinoma. From these findings, we diagnosed this lesion as MCC concurrent with Bowen disease.

Childhood Lichen Planus with Palmoplantar Involvement

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.

A Case of Herpetiform Appearance of Digital Mucous Cysts

A 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.

A Case of Myopericytoma on the Lower Leg

Myopericytoma (MP) is a benign tumor composed of cells that show apparent differentiation towards putative perivascular myoid cells called myopericytes. It arises most commonly in the dermis or subcutaneous tissue of the extremities in adults. The most common presentation is a well-circumscribed, slow-growing painless firm mass. A 45-year-old woman presented with a 2-year history of a painless, slowly growing 0.9×0.7 cm sized firm mass in the subcutaneous tissue of the posterior side of the right lower leg. We presumed this lesion to be an epidermal cyst, pilomatricoma or calcinosis cutis and performed an excisional biopsy. The histologic examination showed that it was composed of spindle-shaped myoid-appearing cells in a concentric arrangement, intimately associated with thin-walled vascular channels. Lesional spindle cells were diffusely positive for smooth muscle actin and were negative for CD34, desmin and S100 protein. From these findings, we diagnosed this lesion as a myopericytoma.