Weber-Christian Disease Treated with Steroid Pulse Therapy / 대한피부과학회지

Weber-Christian disease is an inflammatory disease of subcutaneous fat tissue which is characterized by relapsing, febrile tender nodules and histologically lobular panniculitis. Many cases originally considered as examples of Weber-Christian disease were later found to be other diseases when lobular panniculitis was reclassified, and some authors believe that Weber-Christian disease should no longer be considered as a distinctive entity. However, there are still occasional cases which cannot be diagnosed as other specific diseases but have the characteristic manifestations of Weber-Christian disease, and they attest to the fact that Weber-Christian disease could be a distinctive disease entity. We report here a case of Weber-Christian disease in a 22-year-old female who was treated successfully with steroid pulse therapy.

Cutaneous T Cell Lymphoma, Pleomorphic, Small/medium-sized with Tissue and Blood Eosinophilia / 대한피부과학회지

Cutaneous T cell lymphoma, pleomorphic, small/medium-sized is a primary cutaneous lymphoma that consists of less than 30% of large, pleomorphic T cells and mostly small/medium-sized, plemorphic T cells stained negatively CD30. This entity presents clinically with one or several red-purple nodules without patches & plaques and histopathologically infiltration of small/medium-sized T cells with a dense nodular or diffuse pattern in the dermis. Usually cellular infiltrations consist of atypical lymphocytes without eosinophils, plasma cells and neutrophils but there are cases infiltrated with atypical lymphocytes and eosinophils. We report a case of cutaneous T cell lymphoma, pleomorphic, small/medium-sized in a 55-year-old male who was presented with hard nodules on both elbows, both ankles, left 1st interdigital web, both wrists and both lateral sides of thigh. Histopathological findings showed diffuse and nodular infiltrates with atypical lymphocytes and eosinophils in the dermis, not involving the epidermis. Bone scan, bone marrow biopsy and CT scan taken to find out involvement of other organs showed normal finding except for eosinophilia at bone marrow biopsy.

Extensive Mongolian Spots Associated with Hunter Syndrome / 대한피부과학회지

We report a case of Hunter syndrome with extensive Mongolian spots. A 4-year-old male baby presented with asymptomatic, firm, raised, ivory-colored papules and nodules which coalesced to form ridges in a reticular pattern (pebbling of the skin) and were arranged bilaterally and symmetrically over the scapulae, and lateral aspects of the thighs. Large Mongolian spots were present over the back. He has low intelligence, short stature, a protuberant abdomen with hepatosplenomegaly, and broad hands with clawlike contractures of the distal interphalangeal joints. The result of qualitative analysis of mucopolysaccharride was positive for the dermatan sulfate and heparan sulfate in the urine. Massive mucinous materials which stained positively with alcian blue were observed from collagen fibers throughout the dermis of skin biopsy specimen. Metachromatic granules within fibroblasts stained with toluidine blue were also seen. This case is reported for its rarity and the typical skin lesions, the recognition of which may be helpful in diagnosis and genetic counselling.

Epithelioid Hemangioma Associated with Nevus Flammeus / 대한피부과학회지

Epithelioid hemangioma, the term which was first used by Enzinger and Weiss, is synonymous with angiolymphoid hyperplasia with eosinophilia. It defines a lesion occurring usually in the dermal and subcutaneous tissue of the head and neck, characterized by localized, marginated and relatively symmetrical proliferation of the capillaries around a medium-sized vessel with an epithelioid hyperplasia of endothelial cell. There is a marked inflammatory reaction composed of lymphocytes and eosinophils. We report a case of epithelioid hemangioma associated with nevus flammeus in a 53-year-old male. Histology revealed typical feature of epithelioid hemangioma that proliferation of dilated vessels with prominent endothelial cells surrounded by dense lymphoid cell infiltrates but no eosinophils were identified.

A Case of multiple cutaneous neurofibromas treated by CO2 laser / 대한피부과학회지

Multiple Cutaneous neurofibromas are the most common skin manifestation of Von Recklinghausen neurofibromatosis(NF-1). The CO2 laser is presented as a useful tool for the removal of large numbers of small- or medium-sized cutaneous neurofibromas. Its advantages include high patient satisfaction with the rapid removal of hundreds of neurofibromas, with minimal morbidity and an enhanced appearance. We report a case of multiple cutaneous neurofibromas treated by CO2 laser in a 40 year-old man whom we eliminated 288 cutaneous neurofibromas out of 788 in total. After 3 months of follow up, the lesions were healed with slight erythemas and minimal scars.

Pigmented eccrine poroma with squamous differentiation / 대한피부과학회지

Eccrine poroma is a common adnexal neoplasm that differentiated toward the acrosyringium, the intraepidermal portion of the eccrine sweat duct. This disease appear clinically as skin-colored or brownish nodule, but the lesion can be pigmented when it contains melanocytes and melanin. In that case, it can be very similar to melanoma. Futhermore, eccrine poroma rarely shows squamous differentiation histologically. Nevertheless, it should be differentiaed from basalcell carcinoma and seborrheic keratosis when it shows squamous differentiation. We report a case of pigmented eccrine poroma with squamous differentiation in a 42-year-old male.

Clinical and Histologic Features of Pityriasis Rosea and Pityriasis Lichenoides in Children / 대한피부과학회지

BACKGROUND: Pityriasis rosea is an acute inflammatory dermatosis with an unproven etiology. The typical clinical feature consists of an initial, single herald patch followed by development of smaller, disseminated, papulosquamous, ovoid macules. However, an unusual papular type of pityriasis rosea is relatively common in children and it shows similar clinical characteristics to pityriasis lichenoides. OBJECTIVES: Our purpose was to observe the clinical and histologic features of pityriasis rosea and pityriasis lichenoides, and differentiate two diseases in children. METHODS: Sixteen children with pityriasis rosea and nineteen children with pityriasis lichenoides were investigated clinically and histologically. RESULTS: 1. Clinically, pityriasis rosea may be differentiated from pityriasis lichenoides by the existence of herald patch, short duration, the uniformity of size and shape, and residual pigmentation. 2. Focal parakeratosis, intraepidermal vesicle and papillary derma edema are more common in pityriasis rosea. In contrast, deep dermal infiltration, dyskeratosis, epidermal necrosis and confluent parakeratosis are more common in pityriasis lichenoides. 3. Comparing to the plaque type, a papular type of pityriasis rosea shows more involvement in the extremities with longer duration. Histologically, parakeratosis and papillary dermal edema are less and spongiosis is more commonly observed in papular type. CONCLUSIONS: Pityriasis rosea in children often shows papules especially on the extremities, similar to that of pityriasis lichenoides. However, from the results in this study, we believe that two diseases can be differentiated if both clinical and histologic findings are carefully studied.