Acute exanthematous pustular drug eruption induced by mexiletine

A 56-year-old man developed infiltrated erythemas on the trunk, extremities, and face with marked facial edema, one month after taking mexiletine hydrochloride for his arrhythmia. A number of pustules were also noted on the surface of erythemas on his chest and face. Laboratory examination showed liver dysfunction and hypereosinophilia. The culture from pustules was sterile. Histological examination of the biopsied skin from a pustular lesion revealed a subcorneal abscess, and perivascular infiltration of lymphocytes, mononuclear cells and eosinophils in the upper dermis. The skin lesions and facial edema as well were improved within three weeks by withdrawal of mexiletine hydrochloride. Patch tests with 10% and 20% mexiletine hydrochloride in petrolatum showed positive reaction, however, pustules were not provoked on the tested site. We conclude that pustules, infiltrated erythema and facial edema were the signs of acute exanthematous pustular drug eruption induced by mexiletine hydrochloride.     

Sterile eosinophilic pustulosis (Ofuji)

In recent years there has been an increase in the information available on "eosinophilic pustular folliculitis (Ofuji)". A significant feature of this disease is extensive tissue eosinophilia in association with follicular, intraepidermal or subcorneal pustules containing eosinophils. A review of the current literature reveals that the skin lesions vary widely. Some patients present with pustules on an erythematous base; in others, annular or circinate papulonodular lesions predominate, which may become crusted. Even vesicular lesions have been reported. The extensive tissue eosinophilia itself suggests that the condition is not caused by an exogenous infectious agent. Most data implicate an immunopathological event that is still not well understood.     

特发于手掌部的嗜酸性脓疱性毛囊炎1例

患者男,19岁。左手掌反复出现水疱、脓疱伴瘙痒2月余。体检:左手掌见多处淡红色轻度脱屑性斑疹、斑片,伴有小水疱、脓疱及结痂,右手掌散在多个水疱。外周血和骨髓中嗜酸性粒细胞增多。左手掌皮损组织病理检查示:真皮及皮下组织中大量以嗜酸粒细胞为主的炎细胞浸润。诊断:嗜酸性脓疱性毛囊炎。口服强的松30mg/d后皮损迅速消退。     

Eosinophilic pustular eruption associated with transdermal fentanyl

A 70-year-old man developed a widespread eruption of sterile pustules on normal skin, mainly on the trunk, face and base of the limbs, 2 days after application of a fentanyl-transdermal therapeutic system (fentanyl-TTS) patch. The eruption was not accompanied by fever. The main histopathological finding was an intraepidermal pustule filled almost exclusively with eosinophils. Suspension of the fentanyl-TTS led to rapid and definitive spontaneous regression of the pustules in about 10 days. A similar pustular reaction related temporally to fentanyl-TTS administration had appeared several weeks before the current eruption.     

Eosinophilic pustular folliculitis in infancy: report of two affected brothers

We describe two brothers with eosinophilic pustular folliculitis. Both had recurrent crops of papules and pustules, primarily affecting the scalp. The eruption began in the neonatal period. Both children had a leucocytosis with eosinophilia. Histology revealed folliculitis, with an infiltrate in which eosinophils were predominant. Bacteriological and fungal cultures of pustules were negative. There was a good clinical response to treatment with a topical corticosteroid and dapsone. We review the 28 previously reported cases of eosinophilic pustular folliculitis in infancy. The occurrence of this disorder in brothers suggests that an inherited or contagious factor may be involved in its aetiology.     

A case of sterile transient neonatal pustulosis presenting with large flaccid pustules

We describe a female neonate with large flaccid pustules on her trunk and extremities. Cultures were negative, and a Wright‐stained smear of pustule contents showed numerous eosinophils (>90%). The pustules resolved without leaving pigmentation after 2 weeks. We diagnosed this case as sterile transient neonatal pustulosis.     

Pathergy in atypical eosinophilic pustular folliculitis

A 15-year-old, unmarried female presented to our dermatology department for an intensely pruritic skin rash that had appeared abruptly 3 days earlier. She had a remarkable medical history for a case of allergic rhinitis and several attacks of asthma in her early childhood. The condition waxed and waned initially but had improved in recent years. Physical examination revealed several erythematous plaques, papules studded with scattered pustules having diameters less than 0.3 mm. Conspicuous scratch marks had caused erythematous wheal-like indurations also studded with pustules in a linear distribution across the waist, forearms (Fig. 1), and back (Fig. 2). Discrete papulopustules were present on the face, nape and neck. The patient was otherwise healthy. There were no other symptoms such as fever, malaise, weakness, or lymphadenopathy Laboratory results were normal for hepatic and renal functions, serum electrolytes, glucose, protein, erythrocyte sedimentation rate (8 mm/h), and C-reactive protein (0.355 mg/l). A human immunodeficiency virus (HIV) antibody screen test was negative. Serum was positive for herpes simplex virus (HSV)-1 and HSV-2 IgG (in low titers), but negative for HSV-1 and HSV-2 IgM. White blood cell count revealed leukocytosis (11.2 x 10(3)/l), with a differential count of 68% neutrophils, 27% lymphocytes, and 8% eosinophils. Serum IgA, IgG, and IgM were within normal limits, but the IgE level was elevated (677 mg/dl). Cultures from peripheral blood and pustules were negative. A Tzank smear performed on the pustules showed no multinucleated giant cells. Fungal testing of skin scrapings from the initial lesion site gave negative results. Routine stool tests, including common pathogen and parasite screens, were negative, and urinalysis results were unremarkable. A biopsy specimen obtained from a skin pustule showed subcorneal eosinophilic and neutrophilic pustules in the follicular infundibulum with marked spongiosis of the follicular epithelium. (Fig. 3). There was a moderately dense superficial and deep perivascular mixed inflammatory cell infiltrate comprising eosinophils, neutrophils and lymphocytes. Migration of eosinophils and neutrophils through the vessel wall with variable luminal intramural fibrin deposition, histologically indicative of vasculopathy, was seen. There was concomitant slight perivascular dermal necrosis. (Fig. 4). Based on the clinical presentation and light microscopic findings on biopsy, a diagnosis of eosinophilic pustular folliculitis with pathergy was made. Systemic prednisolone 30 mg in divided doses was given. After 1 week of systemic corticosteroid therapy, the patient's condition was significantly improved and the patient was subsequently discharged. Two months later she had a relapse, upon which corticosteroid therapy was commenced leading to lesional resolution. The foci of eosinophilic folliculitis healed with areas of hyperpigmentation with variable scarring.     

Eosinophilic pustulosis of the scalp in childhood.

BACKGROUND: Among sterile pustulosis in childhood, a pruritic relapsing eosinophilic variant beginning in infancy and located mostly in the scalp was first described as eosinophilic pustular folliculitis in infancy by Lucky and colleagues in 1984. OBJECTIVE: Our purpose is to describe such a condition in five boys and one girl and comment on differential diagnosis and relation with Ofuji's disease. METHOD: This is a clinicopathologic study. RESULTS: All patients had scalp pustules beginning in infancy or early childhood that were unresponsive to antibiotic therapy. Lesions also occurred on other areas but the scalp was the major site of involvement. Although secondary infection was demonstrated in one case, the lesions were primarily sterile. Smears of pustules showed a variable proportion of eosinophils. Histopathologic findings suggested a major role for eosinophils in this disorder because dermal eosinophilia was noted in all patients. The inflammatory pattern was not similar to Ofuji's disease. Transient blood eosinophilia was recorded in five patients. Topical steroids relieved inflammatory episodes. Dapsone was tried in one case with apparent benefit. CONCLUSION: Eosinophilic pustulosis of the scalp in childhood is a self-limited disease that can be relieved by topical steroids.     

Acute generalized exanthematous pustulosis due to clindamycin

Acute generalized exanthematous pustulosis (AGEP) is a rare skin eruption most commonly caused by medications. It is characterized by fever and the acute eruption of non-follicular pustules overlying erythrodermic skin. Histopathology shows subcorneal pustules with a background of dermal edema and spongiosis, leukocytoclastic vasculitis, perivascular eosinophils, and focal necrosis of keratinocytes. Three cases of clindamycin induced AGEP have been reported in the literature. A case of AGEP due to clindamycin is reported in a patient with numerous other drug allergies and without history of psoriasis. Presentation and treatment of AGEP are reviewed.     

Erosive pustular dermatosis of the leg: long-term control with topical tacrolimus

Erosive pustular dermatosis of the leg is an unusual form of sterile pustulosis that typically affects the lower limbs of elderly patients. We report the cases of two women who developed erythematous skin plaques with pustules that coalesced and evolved into erosions and crusted areas. Histology showed epidermal spongiosis with subcorneal pustules and a dermal infiltrate with eosinophils and neutrophils. Lesions were treated with topical clobetasol propionate 0.05% for 10 days followed by topical tacrolimus daily until complete resolution, and then twice weekly for 1 year, without relapse. The response to topical corticosteroids and tacrolimus further support the close relationship with erosive pustular dermatosis of the scalp. Topical therapy with tacrolimus may offer good long-term disease control.     

增殖性脓性皮炎-脓性口炎一例

【摘要】 患者女,58岁,唇、头皮、皱褶部位反复发生红斑、脓疱、增殖性斑块3个月。患者有溃疡性结肠炎病史2年,皮疹发生前肠炎加重。皮肤科检查：双唇弥漫性增厚,上有密集米粒至绿豆大脓疱,部分脓疱融合,唇缘黄色结痂;左颊黏膜有一1.5 cm × 2 cm的增殖性斑块,边缘脓疱呈环状排列;头皮、颈、左腋窝、脐周、左腹股沟可见多发性大小不等的增殖性斑块、糜烂、黄色结痂,中央和边缘有环状排列的水疱和脓疱,皮疹消退后留色素沉着斑;右腋4个新发脓疱。下唇缘皮损组织病理学检查：表皮轻度增厚伴中性粒细胞微脓肿,真皮密集混合性炎症细胞浸润,可见中性粒细胞和嗜酸性粒细胞微脓肿;下唇皮疹边缘正常皮肤直接免疫荧光检查阴性。血常规检查示嗜酸性粒细胞比例增高。脓疱细菌、真菌培养阴性。诊断：增殖性脓性皮炎-脓性口炎。 【关键词】 增殖性脓性皮炎-脓性口炎     

嗜酸性粒细胞性脓疱性毛囊炎1例

嗜酸性粒细胞性脓疱性毛囊炎（EPF）在国内较少报告。本文报告1例EPF。患者男,32岁,右眉弓上方红斑伴瘙痒3个月,病理切片显示：大量嗜酸性粒细胞侵入毛囊外毛根鞘部,形成脓肿。真皮上部也有较多嗜酸性粒细胞,为较典型的经典型EPF。     

A case of acute generalized exanthematous pustulosis (AGEP) possibly induced by iohexol

Acute generalized exanthematous pustulosis (AGEP) is an uncommon disease manifested as an erythematous pustular eruption. It is usually caused by systemic medication. We describe a patient with acute generalized pustular eruption induced by iohexol. A 52-year-old woman developed fever and a generalized pustular eruption on the neck, trunk and extremities three days after taking iohexol. The culture from pustules was sterile. Other systemic and laboratory examinations were normal. A skin biopsy from a lesion on the trunk showed the features of a drug-induced pustular eruption as a subcorneal blister including neutrophils and eosinophils, mild spongiosis, and a sparse infiltrate at neutrophils and eosinophils in the papillary dermis. The patient had no history of psoriasis. The lesions resolved with systemic corticosteroid therapy within one week and did not relapse. According to our investigation, iohexol-induced AGEP has not been previously reported. We present an interesting case.     

TRANSIENT NEONATAL PUSTULAR MELANOSIS

A Mexican-American boy presented at birth with an extensive eruption consisting of 0.5 to 1.0 cm hyperpigmented macules with a distinct peripheral scale involving primarily the forearms, abdomen and lower back (Fig. 1). Rare intact vesicopustules were also identified. There was an unremarkable prenatal history, and the infant was a product of a normal vaginal delivery. With the exception of the skin lesions and moderate hepatosplenomegaly, the physical examination was normal. Gram stains of the pustules showed numerous neutrophils but no bacteria. Bacterial cultures, of the skin and blood, TORCH screen (toxoplasmosis, rubella, cytomegalic virus, and herpes virus) and a VDRL were negative. On the second day of life, the patient developed several pustules with surrounding erythema consistent with erythema toxicum neonatorum. Wright-stained smears of these lesions showed abundant eosinophils. Hepatosplenomegaly resolved by the third day of life and at the time of discharge only hyperpigmented macules persisted. Follow-up visit six weeks later showed no evidence of skin lesions.     

Ofuji's disease with unusual histological features

A 20-year-old lady presented with a 3-month history of an itchy eruption affecting her face and trunk. Erythematous annular plaques with follicular papules and pustules at the margins were present on her face consistent with Ofuji's disease. A perifollicular and perivascular eosinophil-rich infiltrate was seen histologically, with infiltration of the follicular epithelium by eosinophils and formation of eosinophilic microabscesses. An unusual feature was the presence of follicular mucinosis. Treatment with dapsone resulted in an improvement in the eruption after 2 weeks. Ofuji's eosinophilic pustular folliculitis is becoming increasingly frequently recognized, and may not be as rare as initial reports suggested.     

Pemphigus vegetans of the oral cavity

BACKGROUND: Pemphigus vegetans, a variant of pemphigus vulgaris, constitutes a rare form of all pemphigus cases, and oral involvement is common. Two clinical subtypes of pemphigus vegetans exist, characterized initially by flaccid bullae and erosions (Neumann) or pustules (Hallopeau). Both subtypes subsequently develop into hyperpigmented vegetative plaques with pustules and hypertrophic granulation tissue at the periphery. METHODS: We report three cases of pemphigus vegetans with oral manifestations exclusively. Two patients were male aged 30 and 45 years old, respectively, while one was a 51-year-old female. CONCLUSION: Oral lesions in all cases consisted of erosions and whitish, vegetating plaques. The histopathological characteristics were in all cases identical. The spinous cell layer was characterized by intense acanthosis and by the presence of vesicles between the spinous and basal cell layers. Inside the vesicles exudative elements were observed consisting mainly of eosinophils. In the upper lamina propria severe inflammatory reaction was observed. Streptavidin-biotin immunoperoxidase technique showed in all cases intercellular epithelial deposition of IgG and C3.     

Childhood eosinophilic pustulosis

Childhood eosinophilic pustulosis is a rare disease that is characterized by recurrent outbreaks of pruritic pustules and follicular papules. The lesions are sterile and contain masses of eosinophils related to the scalp hair follicle. Because of the good prognosis for these symptoms, conservative treatment with topical corticosteroids is recommended. We present two cases of this disease, describing its clinical course and development over two years.     

Pyostomatitis vegetans

Pyostomatitis vegetans is a rare oral disorder often associated with gastrointestinal and/or cutaneous diseases and sometimes with other disorders. The association of pyostomatitis vegetans with inflammatory bowel disease, particularly ulcerative colitis, is well known and in most cases bowel disease precedes the onset of oral lesions by months or years. A peripheral eosinophilia has been observed in most reported cases. Pyostomatitis vegetans is characterized by erythematous, thickened oral mucosa with multiple pustules and superficial erosions. Histopathologically, it is characterized by intraepithelial and/or subepithelial abscesses containing large numbers of eosinophils. The pathogenesis is as yet unknown although immunological and microbial factors have been suggested as possible aetiological factors. Management of pyostomatitis vegetans consists mainly of immunosuppression using topical or systemic corticosteroids with medical and/or surgical treatment of any underlying inflammatory bowel disease.     

Incontinentia pigmenti Stage 1 is not simply vesiculo-bullous but vesiculo-pustular

Incontinentia pigmenti (IP) is a rare X-linked dominant, male-lethal disorder characterized by pathognomic skin lesions. As described in the literature the typical cutaneous changes follow the pattern of Blaschko's lines and develop in four stages that usually start at birth. Stage 1 is called vesicular, bullous or inflammatory. The vesicles are rapidly filled with eosinophils and thus turn into pustules. Thus, the term “pustular” is relevant to the first phase of IP, and the stage can be considered as “vesiculopustular/inflammatory” to be more precise than “vesicular” or “bullous.”