An observational study of the efficacy of azithromycin in erythema annulare centrifugum.

Erythema annulare centrifugum (EAC) is a form of figurate erythema consequent to a cutaneous hypersensitivity reaction to an underlying agent. In the present study, we aimed to assess the role of oral azithromycin in cases of idiopathic EAC. We performed an open trial of azithromycin in 10 patients with idiopathic EAC. Histopathological examination of biopsies was performed to exclude any alternative diagnosis and to assess the depth of the infiltrate. Patients were administered oral azithromycin 250 mg once daily until clinical resolution of the disease, and followed up regularly to monitor for possible relapse. Histopathological examination of the 10 biopsies revealed superficial pattern in 3, deep pattern in 2 and mixed pattern in the remaining 5. Of the 10 patients, 8 responded to azithromycin 250 mg, with no relapse during follow-up. Oral azithromycin might be a promising therapy in cases of idiopathic EAC. Cases with a superficial pattern respond earlier than cases with a deep pattern.

Dermatopathia Pigmentosa Reticularis with Salzmann's nodular degeneration of cornea: A rare association.

BACKGROUND: Dermatopathia pigmentosa reticularis (DPR) is a very rare autosomal dominant disorder with the diagnostic triad of generalized reticulate hyperpigmentation, noncicatricial alopecia and onychodystrophy. OBJECTIVE: To describe the occurrence of Salzmann's nodular degeneration of cornea with moderate dry eye in a patient with Dermatopathia pigmentosa reticularis. CASE: We present an 11 year old young Indian girl with DPR who had Salzmann's nodular degeneration of cornea with moderate dry eye. She was put on symptomatic treatment and counseled regarding the course of disease, familial nature and avoidance of exposure to sun. CONCLUSION: In a patient of Salzmann`s nodular degeneration with generalized reticulate hyperpigmentation, noncicatricial alopecia and onychodystrophy the diagnosis of DPR must be kept in mind. A multidisciplinary approach is required for the management of such cases.

Bioethical issues of preventing hereditary diseases with late onset in the Sakha Republic (Yakutia).

BACKGROUND: Prenatal diagnosis of congenital and hereditary diseases is a priority for the development of medical technologies in Russia. However, there are not many published research results on bioethical issues of prenatal DNA testing. OBJECTIVE: The main goal of the article is to describe some of the bioethical aspects of prenatal DNA diagnosis of hereditary diseases with late onset in genetic counselling practice in the Sakha Republic (Yakutia) - a far north-eastern region of Russia. METHODS: The methods used in the research are genetic counselling, invasive chorionic villus biopsy procedures, molecular diagnosis, social and demographic characteristics of patients. RESULTS: In 10 years, 48 (76%) pregnant women from families tainted with hereditary spinocerebellar ataxia type 1 and 15 pregnant women from families with myotonic dystrophy have applied for medical and genetic counselling in order to undergo prenatal DNA testing. The average number of applications is 7-8 per year. There are differences in prenatal genetic counselling approaches. CONCLUSION: It is necessary to develop differentiated ethical approaches depending on the mode of inheritance, age of manifestation, and clinical polymorphism of hereditary disease.

'Congenital follicular melanocytic naevi': a more appropriate term for spotted grouped pigmented naevi.

We report two patients with an uncommon form of pigmented naevus consisting of grouped follicular papules. A biopsy taken from the lesions showed multiple naevus cells, predominantly around the hair follicles, with sparing of the eccrine glands. The clinicohistopathological term given for this condition is 'spotted grouped pigmented naevi type I', and has rarely been reported. We discuss the unusual morphology and differential diagnosis of this condition, and suggest that the term 'congenital follicular melanocytic naevi' is more appropriate for this presentation.

Granulomatous cheilitis secondary to tuberculosis in a child.

Granulomatous cheilitis is a chronic granulomatous inflammation of the lips that can be secondary to various etiologies. A few infectious agents including Mycobacterium tuberculosis have been implicated in its etiology. It can be the clinical presentation of a tuberculide resulting from a hypersensitivity reaction to an underlying focus of tuberculosis such as pulmonary tuberculosis. This case report describes a child with granulomatous cheilitis with pulmonary tuberculosis, who responded to anti-tubercular treatment. This is probably the first pediatric case of this rare condition.

An observational analysis of erythromelanosis follicularis faciei et colli.

Erythromelanosis follicularis faciei et colli (EFFC) is characterized by well-demarcated erythema, hyperpigmentation, and follicular papules. Since the orginal description, < 50 patients have been reported in the literature. Five cases of EFFC have been seen in our outpatient department in the past 5 years. All patients except one presented with the classic triad of erythema, brown pigmentation and follicular papules on the face and keratosis pilaris lesions on the trunk. In all cases, there was no family history for related disorders or history of atopy. Histopathological examination in all cases was consistent with the diagnosis of EFFC. Age at presentation ranged from 11 to 19 years, and the male female ratio was 4 : 1. Patients had the classic lesions of keratosis pilaris on the face (5/5), trunk (2/5) and legs (4/5). All patients except one had a background of erythema. One patient (patient 2) had itching and a history of photosensitivity. Of the patients who were on treatment (4/5), complete clinical regression was seen in one patient. To our knowledge, this is the first case series of EFFC from India, and we believe that EFFC is a common but infrequently reported condition.

Classic Kaposi's sarcoma in a Nepalese woman from a purportedly nonendemic area.

Kaposis sarcoma (KS) occurs as four types; classic, endemic, immunosuppression-associated, and AIDS-associated. The presence of KS-associated herpesvirus (human herpesvirus 8) DNA in the tissue samples of 95% of all variants of KS has confirmed its role in the aetiopathogenesis of KS. It is now believed that these clinical variants most likely represent different manifestations of the same pathological process. Our case represents the first case of classic KS from our region.

Giant combined dermatofibroma with satellitosis.

An unusual giant combined dermatofibroma is reported in a 34-year-old man who presented with skin-coloured swellings on the medial aspect of the left scapula. The plaque was well defined, reddish-brown and 25-30 cm in diameter. It was also tender and indurated. Several similar smaller lesions (satellites) were present around the plaque. Light microscopy of an incisional biopsy from the main lesion showed architectural features of a deep penetrating type of dermatofibroma, with xanthomatous aggregates, myxoid changes and probable myofibroblastic differentiation. Our case represents an extraordinary example of giant combined dermatofibroma with satellitosis. Despite its benign nature, a wide excision is contemplated because of its unsightly appearance and physical discomfort.

Tuberculosis of the vulva masquerading as a sexually transmitted disease.

Tuberculosis of the vulva is a rare condition usually seen by a gynecologist. We report a case of chronic ulcer on the vulva of four months duration, which, on detailed investigation, turned out to be a case of primary inoculation tuberculosis. The patient was subsequently put on antitubercular therapy with a good the therapeutic response. The rarity of this presentation in dermato-venereology is emphasized.

Linear and whorled nevoid hypermelanosis.

Linear and whorled nevoid hypermelanosis (LWNHM) is a reticulate pigmentary disorder with a sporadic occurrence, representing genetic mosaicism. It is characterised by hyperpigmented macules in a reticulate pattern along Blaschko's lines, sparing the mucous membranes and stabilising after one to two years. It may be associated with various neurological abnormalities. The disorder may resemble incontinentia pigmenti, epidermal nevus, or zebra-like hyperpigmentation clinically. We report LWMNHM in a 15-year-old girl with progressively increasing streaks of reticulate hyperpigmented macules arranged in a whorled pattern over the trunk and extremities, which appeared soon after birth. There was no history of any preceding eruption or any associated systemic abnormality. Histopathological examination revealed basal cell hyperpigmentation without any pigmentary incontinence. CT scan of the brain was normal.