A kaposiform haemangioendothelioma successfully treated with topical tacrolimus and compression bandaging.

We report a patient with kaposiform haemangioendothelioma successfully treated with topical tacrolimus and compression bandaging.

The spectrum of manifestations in desmoplakin gene (DSP) spectrin repeat 6 domain mutations: Immunophenotyping and response to ustekinumab.

BACKGROUND: The immune abnormalities underlying the ichthyoses are poorly understood. OBJECTIVE: To determine the immunophenotype of an ichthyosis resulting from mutations in the spectrin repeat 6 (SR6) domain of desmoplakin gene (DSP) and target therapy on the basis of molecular pathogenesis. METHODS: Immunophenotyping was performed by using the blood and skin of a girl with SR6 region DSP mutations causing erythroderma/ichthyosis and cardiomyopathy. RESULTS: On the basis of the discovery of T helper 1 and T helper 17/interleukin 23 skewing in the skin and T helper 17/interleukin 22 skewing in blood, ustekinumab therapy was initiated. Ustekinumab was also administered to a boy with an SR6 region DSP mutation and ichthyosis without cardiomyopathy. Both children responded despite previous poor responses to immunosuppressants and retinoids. LIMITATIONS: Small number of patients and immunophenotyping in only 1 patient. CONCLUSION: An understanding of the molecular basis of inflammation in rare cutaneous disorders can lead to targeted therapy, which promises to be more beneficial than broad immunosuppressants.

Genital psoriasis is associated with significant impairment in quality of life and sexual functioning.

BACKGROUND: Genital involvement has significant psychosexual implications for psoriasis patients. OBJECTIVE: This study was designed to ascertain factors associated with the development of genital psoriasis and its impact on quality of life and sexual functioning. METHODS: This was an observational, multicenter study of 354 consecutive psoriasis patients. RESULTS: One hundred thirty-four patients (38%) had current genital involvement while 224 (63%) had a current and/or previous history of genital involvement. Eighty-seven percent reported itch, 39% pain, 42% dyspareunia, 32% a worsening of their genital psoriasis after intercourse, and 43% a decreased frequency of intercourse. Younger age of onset of psoriasis, male sex, more severe disease, and involvement of the scalp, flexures, and nails were associated with the presence of genital disease. There was no association with circumcision or obesity. Patients with genital psoriasis had more impairment in quality of life and sexual health as determined by the Dermatology Life Quality Index (P < .0001), the Center for Epidemiological Studies-Depression Scale (P = .01), and the Relationship and Sexuality Scale (P < .0001). LIMITATIONS: This was a descriptive study from 2 tertiary referral centers where patients were likely to have more severe psoriasis. CONCLUSION: This study highlights the high prevalence of genital psoriasis and its profound impact on quality of life and sexual health.

A retrospective analysis of infantile haemangioma referrals: a comparison with the IH European Task Force 'Infantile Haemangioma Referral Score'.

BACKGROUND: Infantile haemangiomas (IH) are common vascular tumours of infancy that can have significant complications. The IH European Task Force developed the IH Referral Score (IHReS) to help non-specialists quickly identify IH that need to be referred to optimise outcomes in high-risk lesions. AIMS: The aim of this study was to assess the quality of IH referrals to a national care centre and compare the IH referred to the IHReS tool. METHODS: This was a retrospective cross-sectional study that collected data from a random sample of 56 IH patients over a 13-month period. RESULTS: Less than 10% included a photograph (8.93%) and a minority of referrals included if there were complications including ulceration (1.79%) or function threatening (8.93%). Three-quarters of patients satisfied the IHReS criteria. CONCLUSION: The IHReS screening tool is a convenient and efficient resource for non-experts to identify children who require referral to a specialist centre.

Pyoderma gangrenosum induced by episiotomy.

We present a patient who developed genital ulceration within hours following episiotomy procedure during a normal vaginal delivery. This was initially treated by the gynaecology and medical team as cellulitis with no improvement. A diagnosis of pyoderma gangrenosum (PG) was made by the dermatology team 12 days later. On further investigation, she was found to be hepatitis C positive. We report this case to highlight the phenomenon of pathergy and frequent misdiagnosis of PG by other medical teams. When a postsurgical wound is not healing despite relevant systemic treatment, the clinician should suspect PG as an early diagnosis and treatment is crucial.