The efficacy of minocycline in inflammatory dermatoses: a case of prurigo pigmentosa of prepubescent onset in Western world

We present a 21‐year‐old Italian girl with an 8‐year history of missed diagnosed prurigo pigmentosa (PP) successfully treated with short monotherapy with minocycline. PP is an inflammatory disease characterized by recurrent pruritic erythematous papules followed by reticular hyperpigmentation usually located on the trunk. About 300 cases of PP have been described mainly in Japan, whereas only few cases have been reported in Italy. This report shows that minocycline is rapidly effective probably through its ability to scavenge reactive oxygen species and to inhibit the chemotaxis and neutrophil function. Other than its ethnic rarity, this case is very interesting because it is the third case of PP in Caucasian patient with prepubescent onset.     

Bullous prurigo pigmentosa

Prurigo pigmentosa is a rare inflammatory skin disease of unknown etiology, characterized by recurrent, symmetrical, pruritic, erythematous papules resulting in gross reticular hyperpigmentation. The rash occurs mainly on the back, the chest and the nape of the neck. While PP is observed rather frequently in Japan, only a few cases have come to notice in other countries. Vesicular or bullous forms have been reported only rarely. The differential diagnosis includes lichen pigmentosus, pigmented contact dermatitis, confluent and reticulated papillomatosis of Gougerot and Carteaud, dermatitis herpetiformis and bullous lichen ruber planus.This case report concerns a young Caucasian patient with prurigo pigmentosa, in whom predominantly vesicular, but also bullous manifestations appeared on an existing maculopapular eruption on the trunk.     

Prurigo Pigmentosa after a Strict Ketogenic Diet

Prurigo pigmentosa (PP) is a rare inflammatory dermatosis of unknown cause characterized by a predominantly truncal eruption of pruritic erythematous papules in a reticular pattern, resolving with hyperpigmentation. PP is twice as common in girls and women, and the mean age at onset is 25 years. Diagnosis of PP is challenging and is aided by characteristic histopathologic findings. We report a case of PP in a 17‐year‐old white boy. The eruption arose during strict adherence to a ketogenic diet.     

色素性痒疹10例临床分析

对2016年8月至2018年8月我院10例（男1例，女9例）色素性痒疹的临床资料进行回顾性分析。患者平均发病年龄26.42岁。1例早期患者，皮损表现为荨麻疹样的丘疹、斑块；5例进展期患者，皮损表现为网状分布的红色丘疹；2例恢复期患者，皮损表现为上覆痂皮的丘疹伴网状棕色斑疹；2例晚期患者，皮损仅表现为网状色素沉着。 10例患者中5例患者在发病前有饮食改变。1例合并I型糖尿病。1例患者衣物摩擦后皮损加重。1例月经前期皮损加重。6例患者检测了尿酮体，其中3例患者尿酮体升高。 10例患者确诊后给予口服米诺环素100 mg/天口服，疗程7~15天，8例患者皮损消退。     

Prurigo pigmentosa: a clinicopathologic study of 16 cases

Background Prurigo pigmentosa is a rare inflammatory disease of unknown origin. It is characterized by the sudden onset of pruritic erythematous papules, usually involving the trunk and neck, which coalesce to form reticulated, mottled patches. Methods We studied 16 patients with prurigo pigmentosa. The patients were selected from those attending the outpatient Department of Dermatology at the Kyung Hee University Hospital from January 2002 to January 2010. All clinical information was retrospectively collected from medical records. The serum concentrations of ketones (acetoacetic acid, 3‐hydroxybutyrate acid [3‐OHBA]) were examined in four patients, and a test for ketone in the urine was performed in 10 patients. Results The age at the time of diagnosis ranged from 18 to 36 years (mean age: 23.5 years), and the female : male ratio was 14 : 2. Skin lesions were almost always characterized by recurrent pruritic erythematous papules that had resolved, leaving a peculiar, reticulate hyperpigmentation. Eight of 16 patients showed a chronological relationship between a prurigo pigmentosa appearance of skin lesions and dieting or fasting. Histopathological findings were either of fully developed lesions (4/16) or late lesions (12/16). Most patients responded well to minocycline treatment. Ketosis was observed in six patients. Conclusions In conclusion, we propose that ketosis was caused by fasting, and that diet may contribute to the pathogenesis of prurigo pigmentosa. Thus, physicians need to warn that excessive fasting can cause prurigo pigmentosa.     

口服阿莫西林后发生天疱疮

报告1例口服阿莫西林后发生天疱疮的病例。患者男,40岁。因全身出现红斑、水疱1个月后就诊。1个月前患者全身皮肤出现散在黄豆大红斑、瘙痒,2d后在红斑和正常皮肤上出现水疱,水疱破裂后干燥结痂,但仍有新发水疱,皮损多分布在胸、背、颈部和腋下。经口服泼尼松20mg/d治疗2周后,皮损基本消退,5d前全身又出现红斑和水疱,皮损瘙痒。患者2次发病前均有口服阿莫西林史。皮肤科检查:躯干和四肢可见大小不一的散在水肿性红斑,部分红斑上可见松弛性水疱,尼氏征阳性,皮损组织病理检查示表皮内水疱和棘刺松解细胞,免疫组化组织病理示在表皮内IgG、C3呈网状沉积。体外γ干扰素释放实验阳性。最终诊断为药物诱发的天疱疮。     

A Case of Palmar Lichen Nitidus Presenting as a Clinical Feature of Pompholyx

Lichen nitidus (LN) is an uncommon chronic eruption of an unknown cause, and it is characterized by tiny, discrete, flesh-colored papules. The sites of predilection are the genitalia, trunk and extremities. Unilateral palmar involvement with pruritus is infrequent. We report here on a case of LN confined to the right palm, and the patient presented with multiple, pruritic, erythematous to flesh-colored, tiny papules and vesicles that mimicked pompholyx. The histopathological examination of a skin biopsy specimen showed the typical findings of LN.     

Prurigo pigmentosa

BACKGROUND: Prurigo pigmentosa is a rare inflammatory dermatosis of unknown etiology characterized by recurrent, pruritic erythematous papules and gross reticulate hyperpigmentation. It is seen most commonly among young adult Japanese females. Only 20 cases have been described outside Japan. METHODS: We report two female, Turkish patients aged 20 and 26 years who had a pruritic rash with the characteristic clinical appearance and supportive histopathology of prurigo pigmentosa. RESULTS: They were successfully treated with minocycline and doxycycline. CONCLUSIONS: Prurigo pigmentosa is a relatively new clinical entity, and we believe that a more widespread knowledge of this disease will lessen its misdiagnosis. We find it noteworthy to point out that there may be a predisposition to prurigo pigmentosa amongst the Turkish and Sicilian populations.     

Prurigo pigmentosa in a patient with primary biliary cirrhosis and Sjögren syndrome

A 44‐year‐old Japanese woman suddenly developed severely pruritic erythematous papules on her trunk in a symmetrical distribution. Biopsy specimens showed the typical histopathological findings of prurigo pigmentosa. She had had recurrent episodes of high fever spikes for several years, and lost 10 kg in the last year. She was diagnosed as primary biliary cirrhosis (PBC) associated with subclinical Sjögren syndrome (SjS). Predonisolone (60 mg/day) for two weeks was effective for the PBC and fever, but not for the prurigo pigmentosa. PBC may be involved in the pathogenesis of this rare skin disease.     

Tinea versicolor mimicking pityriasis rubra pilaris

Tinea versicolor is a common noninvasive cutaneous fungal disease. We recount a case of tinea versicolor that mimicked type I (classic adult) pityriasis rubra pilaris. A 54-year-old white man reported a 20-year history of a recurrent pruritic eruption that had marginally improved with use of selenium sulfide shampoo and treatment with oral antihistamines. Results of a skin examination revealed erythematous plaques; islands of spared skin; and follicular erythematous keratotic papules on the trunk, shoulders, and upper arms. A lesion was scraped to obtain skin scales for potassium hydroxide staining. Examination of the stained samples revealed the characteristic "spaghetti and meatballs," confirming the diagnosis.     

Prurigo pigmentosa (Nagashima) associated with anorexia nervosa

Prurigo pigmentosa (Nagashima), which is not rare in Japan, is an inflammatory disease of unknown aetiology. The characteristic lesions are pruritic red papules, superseded by reticular hyperpigmentation, characteristically on the back, neck, and chest. ¹ We now report a case of prurigo pigmentosa associated with anorexia nervosa. These findings suggest that the ketosis produced by anorexia nervosa may well contribute to the pathogenesis of prurigo pigmentosa.     

Disseminated angiolymphoid hyperplasia with eosinophilia: a case report

A 37-year-old Cantonese man presented with pruritic erythematous papules and nodules on his face, limbs, and trunk that had been present for 10 years and aggravated for 2 years. More nodules were noticed where the skin was scratched and traumatized. The lesions were alleviated temporarily, but they did not subside entirely without therapy. The lesions responded to treatment with intravenous dexamethasone. Histopathology results indicated angiolymphoid hyperplasia with eosinophilia (ALHE), and the patient was diagnosed with disseminated ALHE (DALHE). His lesions ameliorated after treatment with prednisone.     

Prurigo pigmentosa: not an uncommon disease in the Turkish population

BACKGROUND: Prurigo pigmentosa (PP) is a rare inflammatory dermatosis of unknown etiology. It is characterized by erythematous papules of the trunk that resolve leaving a reticulate hyperpigmentation. The original report from Japan in 1971 was followed by new cases, mostly of Japanese origin. METHODS: We scrutinized PP cases diagnosed in our clinic and reviewed the English, German, French, and Turkish language literature. RESULTS: We report six additional Turkish patients. We found more than 40 cases reported from various countries outside Japan. The patients had different racial origins. The number of Turkish patients reported in recent years is higher than the number of other non-Japanese patients reported. CONCLUSIONS: The disease is not uncommon in the Turkish population. It may be underestimated in other countries with Caucasian populations. Therapeutic results with tetracycline and doxycycline was successful in our patients.     

Erythematous plaque variant of transient acantholytic dermatosis

Transient acantholytic dermatosis (TAD) is a primary cutaneous disorder of unknown cause that was first described by Grover in 1970. The common eruptions of TAD are self-limited pruritic papules and papulovesicules distributed primarily on the trunk and extremities. We report a case of an erythematous plaque variant of this skin disorder.     

Persistent pruritic papules and plaques associated with adult‐onset Still's disease: Report of six cases

Typical skin rash, which appears and disappears along with respective rise and fall of fever, is well‐known, and included as one of the major criteria of adult‐onset Still's disease (AOSD) (Yamaguchi's criteria). In addition, various skin lesions are occasionally observed in association with AOSD. Persistent pruritic eruptions present with some clinical features, such as urticarial erythema, flagellate erythema, erythematous, slightly scaly or crusted papules, and/or plaques on the trunk and extremities. These lesions show unique histological features such as dyskeratosis with a peculiar, distinctive distribution in the upper epidermis and cornified layers with focal hyperkeratosis. We describe herein six cases of AOSD, which presented with skin lesions of persistent pruritic papules and plaques. All six cases were female, and three of them were elderly women. The patients presented with linear erythematous streaks, scaly erythema, keratotic papules, infiltrative plaques and irregular coalesced erythemas. By contrast, histological features were characteristic, and dyskeratotic cells were found in the horny layers as well as in the upper layers of the epidermis. Persistent pruritic eruption is an important cutaneous sign for the diagnosis of AOSD.     

Zileuton, an oral 5-lipoxygenase inhibitor, directly reduces sebum production

BACKGROUND: Zileuton, a 5-lipoxygenase inhibitor, reduces the number of inflammatory lesions in moderate acne and inhibits the synthesis of sebaceous lipids. OBJECTIVE: To detect whether zileuton directly reduces sebum synthesis. METHODS: A 40-year-old female with mild disseminated sebaceous gland hyperplasia and seborrhea was treated with zileuton 4 x 600 mg/day over 2 weeks, was followed-up for 6 weeks after discontinuation of zileuton and was re-treated with low-dose isotretinoin 10 mg/2nd day over 5 weeks. Casual skin surface lipids and sebum synthesis were determined. RESULTS: Under treatment with zileuton increased casual skin surface lipids were normalized and synthesis of facial sebum was decreased. Six weeks after discontinuation of treatment casual skin surface lipids were increased again and synthesis of sebum returned to baseline. Subsequent low-dose isotretinoin treatment led to similar changes of casual skin surface lipids and sebum synthesis with zileuton already after 2 weeks. CONCLUSION: Zileuton directly inhibits sebum synthesis in a transient manner with a potency similar to low-dose isotretinoin at least in our patient.     

Atypical variant of galli-galli disease (grover-like eruption with lentiginous freckling) in a liver transplant patient

Galli-Galli disease is considered as a rare variant of Dowling-Degos disease, sharing the same clinical features of reticulate hyperpigmentation of the flexures. Histopathologically, there is acantholysis and suparabasal lacunae. Grover disease (transient acantholytic dermatosis) is a transient dermatosis which clinically presents as an eruption of erythematous excoriated papules located on the trunk and histologically with dyskeratosis and acantholysis. Grover disease has occasionally been reported in patients with chronic renal failure, human immunodeficiency virus infection, hematological malignancies, bone marrow allotransplantation, and renal transplantation. We report herein a case of atypical variant of Galli-Galli disease occurring in a liver transplant patient in whom the leading sign of reticulate hyperpigmentation of the large flexures was lacking. Instead, Grover-like, erythematous, keratotic, excoriated papules and lentigo-like macules with predilection for the trunk were found. Histopathology of both, an excoriated papule and a lentigo-like macule revealed the diagnostic features of Galli-Galli disease namely, elongated, pigmented, finger-like rete ridges, intraepidermal lacunae and acantholysis. However, serial sections of the lentigo-like macule were needed to reveal some suprabasal and subcorneal lacunae with minimal acantholysis. Thus, a good clinicopathological correlation is essential to make the right diagnosis of Galli-Galli disease. Although Grover disease has been described in the setting of solid organ transplantation and immunosuppression, this is the first report of an atypical variant of Galli-Galli disease occurring in a liver transplant patient.     

Keratosis lichenoides chronica: report of a case developing after erythroderma

A 66-year-old male presented with keratosis lichenoides chronica after a presumed drug-induced erythroderma. After resolution of the erythroderma, slightly scaly erythematous and violaceous papules in a reticular arrangement over the trunk and limbs developed in association with hoarseness, palmoplantar keratoderma, onycholysis and subungual keratosis. Histology from a lichenoid lesion showed pseudo-epitheliomatous hyperplasia, hyperorthokeratosis, parakeratosis, dyskeratosis, neutrophil exocytosis and focal vacuolar degeneration of the basal layer of the epidermis. There was a band-like chronic inflammatory infiltrate in the upper dermis. The skin improved with prednisone 40 mg/day for 15 days, leaving atrophic hypopigmented scars. A diagnosis of keratosis lichenoides chronica was made.     

Treatment of idiopathic prurigo nodularis in Taiwanese patients with low-dose thalidomide

Prurigo nodularis is an intensely pruritic dermatosis characterized by lichenified and excoriated papules and nodules. The course of prurigo nodularis is often chronic, and some patients respond very poorly to the standard therapeutic modalities. Because the pathogenesis of this disease remains obscure, the treatment of prurigo nodularis can be disappointing and frustrating for both the patients and physicians. Thalidomide, a tumor necrosis factor-alpha antagonist, has been suggested as an alternative treatment option for recalcitrant prurigo nodularis. In the past, the regimen for treatment of prurigo nodularis often required thalidomide at 200 mg/day. We recruited patients with intractable prurigo nodularis and treated them with low-dose thalidomide. Six patients with idiopathic prurigo nodularis were successfully treated with low-dose thalidomide (50-100 mg/day) without clinical development of peripheral neuropathy. In summary, our preliminary results suggest that low-dose thalidomide may be a safe and effective treatment option for patients with recalcitrant idiopathic prurigo nodularis.     

Prurigo pigmentosa in Korea: clinicopathological study

Background Prurigo pigmentosa (PP) is an inflammatory dermatosis characterized by recurrent pruritic erythematous papules, mainly located on the trunk. It was first described by Nagashima in 1971 in Japan. Since then, more than 300 cases have been reported in Japan, but reports from other parts of the world are quite rare. Materials and methods We studied clinical and histopathological data from six patients with PP diagnosed in our hospital and 43 patients (18 reports) who were diagnosed with PP in Korea between 1988 and 2008. Results The number of Korean patients reported in recent years is higher than the number of other non‐Japanese patients reported. Clinicopathological characteristics in Korean patients were not significantly different from those previously reported. Therapeutic results with minocycline were successful in our patients. Conclusions We suspect that PP is not uncommon in Korea, and the disease may be underestimated. Strict restriction of diet as well as known associated factors like wet condition are suggested as one of the important factors contributing to the occurrence of PP in Korea.