Pityriasis lichenoides et varioriformis acuta (PLEVA), also known as Mucha-Habermann disease, is an uncommon, idiopathic, acquired dermatosis characterised by erythematous and scaly papules that are often accompanied by haemorrhagic and papulonecrotic lesions. The patient's general health is usually not affected. Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare variant of PLEVA that was first described by Degos et al. in 1966 (1). FUMHD is more destructive and is associated with high fever, systemic symptoms and the development of large coalescent skin necroses which may lead to death. Here we report a young boy with FUMHD successfully treated with systemic corticosteroid. CASE REPORTA 7-year-old Japanese boy presented to our clinic in October 2008 with scattered eruption on the trunk and limbs. Approximately 20 days before consultation, he noticed an egg-sized red macule on his lower back that was preceded by a sore throat and nasal congestion. Next, scattered red eruptions suddenly appeared on his trunk and limbs within 2 weeks and were accompanied by a persistent high fever. The patient's family and past history were unremarkable. Physical examination revealed numerous discrete, bean-sized, oval-shaped erythemas and papules with central ulcers and necrotic crusts distributed over the patient's face, neck, trunk, and extremities (Fig. 1A). The oral and genital mucosae were not affected. Laboratory examinations including haemogram, biochemistry studies, C-reactive protein analysis, urine analysis, and chest X-ray were all within normal limits. A fungal test and skin bacterial cultures of the lesions were negative. Histological examination of an erythema on the forearm revealed hyperkeratosis, acanthosis, necrotic keratinocytes, and degeneration of the basal layer. Numerous mononuclear cells were infiltrated in the epidermis. There were superficial and deep patchy lichenoid lymphohistiocytic infiltrations that obscured the dermo-epidermal interface in the dermis. There were no lymphocytes with atypia (Fig. 1B). Based on the clinico-pathological findings, the diagnosis of FUMHD was established.Oral clarithromycin and potassium iodide treatment were initiated, but the skin lesions and high fever did not improve. Three days later, oral prednisolone (20 mg/ day) was initiated. Most of the necrotic papules disappeared within a week, and the necrotic lesions were healed with scar formation within 3 weeks. The dose of prednisolone was tapered and ended within 2 weeks. No recurrence was observed, and the patient was in good health at a follow-up, approximately 5 years later. DISCUSSIONFUMHD usually begins as typical PLEVA, as was observed in our case, and rapidly evolves to an ulceronecrotic form with haemorrhagic crusts in 2 to 6 weeks. Oral, genital, and conjunctival mucosae can be affected. Associated symptoms include high fever (up to 40°C), malaise, myalgia, arthralgia, gastrointestinal and cen-
We present a 79-year-old man who suffered from extramammary Paget's disease (EMPD) with bowenoid histological features accompanied by an ectopic EMPD lesion on his abdomen. He had had an erythematous plaque on his genital region for three years. Based on a biopsy specimen, he was referred to our hospital with the histological diagnosis of Bowen's disease. The histological findings of the genital lesion obtained by surgical resection showed typical areas of Paget's cells adjacent to areas characteristic of Bowen's disease. Immunohistochemical findings showed CEA and CK7 positive tumor cells in both areas, so the atypical cells showing the bowenoid pattern could be regarded as tumor cells of Paget's disease. Immunohistochemical staining for CEA and CK7 along with multiple biopsies can be helpful in making the diagnosis of Paget's disease with bowenoid histologic features.
Acne fulminans (AF) is a rare condition and the most severe form of acne. It is characterized by the sudden onset of painful and ulcerative pustules and systemic symptoms including high fever and polyarthralgia. Response to ordinary antibiotic agents is poor, and the treatment most oflen recommended is systemic corticosteroids. We report here a case of a young Japanese man with acne fulminans presenting with severe pustules and pyoderma gangrenosum (PG)-like ulcérations. CASE REPORTA 20-year-old Japanese man was referred to our hospital in February 2005, due to a 3-week history of painful erythematous pustular eruptions with high fever. Neither arthralgia nor myalgia were noted. He had been diagnosed as having acne vulgaris for the past 5 years and had sometimes been administered antibiotics such as minocycline. He was otherwise healthy and had no remarkable family history. Neither intensive physical training nor steroid use before the onset ofthe disease were seen.Physical examination revealed multiple follicular pustules and reddish papulonodular eruptions with crusts on his scalp and face (Fig, 1 a). Ulcerated nodules were scattered on the nape and extremities. Walnut-sized ulcerative erythematous plaques with vegetative surface and elevated edges were observed on the extremities (Fig, lb). These lesions resembled PG, A high temperature up to 38,7°C was noted.Abnormal laboratory findings included an increase in leukocytes (16,3 X lOVmm^ and neutrophils (84,3%), C-reactive protein levels were moderately high at 5,0 mg/dl. The complement value was high: CH^^,, > 60 U/ml, Erythrocyte count, haemoglobin value and platelet count were normal. Anti-nuclear antibodies and rheumatoid factor were negative. The serum protein fractions, and liver and renal function, were normal or negative. Bacterial and fungal cultures from the skin lesions were negative. X-ray ofthe chest, vertebrae, ileosacral joints, wrist and finger joints showed no abnormality, A technetium-99 scintigram revealed no abnormal accumulation. Colon fiberscopy was not performed, but the absence of abdominal pain, negative of occult blood of stools suggested the absence of infiammatory bowel disease, A skin biopsy was performed from the ulcerative nodule on his upper back. The epidermis was slightly hypertrophie with a dense crust. In the dermis, a mixed infiltration of polymorphonuclear cells and lymphocytes was observed around the blood vessels and appendages, A massive accumulation of inflammatory cells was seen in and around the hair follicles (Fig, 2a), Peri-and intrafollicular infiltration of inflammatory cells consisted of polymorphonuclear cells and lymphocytes and destruction of the hair follicles was prominent. Numerous giant cells were also observed. No necrotizing vasculitis was noted (Fig, 2b, 2c), Oral administration of prednisolone 40 mg/day and intravenous injection of minocycline rapidly improved the symptoms. The dose of prednisolone was tapered to 10 mg/day in one month, and the skin lesions healed completely with scarring.Two months la...
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