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Summary: These guidelines for the management of congenital ichthyoses have been developed by a multidisciplinary group of European experts following a systematic review of the current literature, an expert conference held in Toulouse in 2016, and a consensus on the discussions. These guidelines summarize evidence and expert‐based recommendations and intend to help clinicians with the management of these rare and often complex diseases. These guidelines comprise two sections. This is part two, covering the management of complications and the particularities of some forms of congenital ichthyosis. What's already known about this topic? Various symptomatic treatment options exist for congenital ichthyoses, but there are no European guidelines. What does this study add? These European guidelines for the management of congenital ichthyosis may help to improve outcomes and quality of life for patients. Linked Comment:Akiyama. Br J Dermatol 2019; 180:449–450. Plain language summary available online [ABSTRACT FROM AUTHOR]

Darier disease is a rare autosomal dominant genodermatosis that initially first presents in adolescence with scaly reddish brown keratotic papules and plaques with a seborrheic and intertriginous distribution. The absence of specific targeted medications complicates the treatment process, and managing resistant cases can prove challenging due to recurrent exacerbations that may result in serious complications such as secondary bacterial and viral infections. Treatments of choice include antiseptics, topical corticosteroids, and systemic retinoids, mainly acitretin and isotretinoin. We report the case of a female patient with Darier disease that was unsuccessfully treated with acitretin and isotretinoin but showed significant improvement with alitretinoin. Previous reports on the efficacy of alitretinoin in Darier disease are reviewed.

Tinea genitalis is a relatively new entity of dermatophyte infection, observed mainly in young adults. By definition, it is localized on the mons pubis and on the labia in women, and on the penile shaft in men. It has been described as a "lifestyle disease" and potentially sexually transmitted disease. We report the case of a 35-year-old patient, an immigrant woman, with tinea genitalis profunda, presenting with painful deep infiltrative papules and plaques, purulent inflammation, and signs of secondary impetiginization. Concomitantly, tinea corporis, tinea faciei, tinea colli, and tinea capitis were diagnosed. Her skin lesions developed over an approximately 2-month period. The zoophilic dermatophyte Trichophyton mentagrophytes was cultivated from the pubogenital lesions, as well as Escherichia coli and Klebsiella pneumoniae. The patient was treated systemically with terbinafine, antibiotics, and short-term corticosteroid, and topically with antimycotic and antibiotic cream. During almost 3 weeks of hospitalization, satisfactory improvement was achieved. A literature review with new clinical and epidemiological observations is presented for this rare type of tinea, which poses a diagnostic and treatment challenge.

Dystrophic epidermolysis bullosa inversa is a very rare subtype of inherited dystrophic epidermolysis bullosa with a unique clinical manifestation. Generalized blistering in the neonatal period and in early infancy improves with age, with lesions becoming restricted to intertriginous areas, axial parts of the trunk, and mucous membranes. In contrast to other variants of dystrophic epidermolysis bullosa, the inverse type has a more favorable prognosis. We present a case of a 45-year-old female patient with dystrophic epidermolysis bullosa inversa, diagnosed in adulthood based on typical clinical presentation, transmission electron microscopic findings, and genetic analysis. Additionally, genetic analysis revealed that the patient also suffered from Charcot-Marie-Tooth disease, a hereditary motor and sensory neuropathy. To our knowledge, the coexistence of these two genetic diseases has not been reported so far. We describe clinical and genetic findings in the patient and review previous reports on dystrophic epidermolysis bullosa inversa. A possible temperature-related pathophysiology for the peculiar clinical manifestation is discussed.

Introduction: Inherited epidermolysis bullosa (EB) is a heterogeneous group of rare genetic skin disorders characterized by fragility of the skin and mucous membranes. The prevalence of all types of EB is estimated at approximately 11 per million, based on recent data from the American National Epidermolysis Bullosa Registry., Methods: A national registry of EB has not yet been established in Slovenia. Because all cases of EB are diagnosed and treated at our department, we have collected data on all known cases of EB in Slovenia., Results: Based on our data, the prevalence of all EB types in Slovenia is about 20 per million. As of December 2020, our data consist of 29 EB simplex, three junctional EB, 10 dominant dystrophic EB, and four recessive dystrophic EB patients., Conclusions: The prevalence of all EB types in Slovenia is higher compared to the estimated prevalence in the United States. The multidisciplinary care of EB patients in Slovenia has been developed based on patients' needs, including a wide group of various specialists, and it has been adapted to the resources and treatment options available. This article also reviews the up-to-date classification and diagnostic protocol for EB, and international recommendations for interdisciplinary patient care.

Hand, foot, and mouth disease is a highly contagious disease that predominantly affects children under the age of five. The illness is much less common in adults and often presents with atypical skin lesions. Here we describe the case of a 19-year-old healthy, immunocompetent male patient with atypical clinical presentation, with a macular rash on the scalp, palms, soles, and shins, and with minimal lesions in the oral cavity. The diagnosis of hand, foot, and mouth disease was confirmed by isolation of coxsackievirus A6 from a vesicle smearobtained on the right sole. The skin lesions resolved spontaneously in 1 week. This case emphasizes the underestimated fact that hand, foot, and mouth disease can also occur in adults, in either typical or frequently atypical form, and it highlights the role of coxsackievirus A6. Together with the case presentation, a review of the literature describing the disease in adults is presented.

Microsporum gypseum complex is a group of geophillic dermatophytes with a worldwide distribution and is a rare cause of dermatomycoses in humans. The infection most commonly presents as tinea corporis, with some geographical and occupational variations. We studied M. gypseum complex infections in patients examined in the Mycological Laboratory of the Department of Dermatovenereology, University Medical Centre Ljubljana, during the period 2000-2015. Diagnosis was confirmed by mycological examination. Skin scales were examined by direct microscopy and cultivated on Sabouraud glucose agar. A total of 226 patients were identified, representing 1.5% of all dermatophyte infections during the study period. Tinea corporis was diagnosed in majority of patients, followed by tinea manus, tinea faciei, tinea inguinalis and tinea pedis. Tinea capitis was observed in three and onychomycosis in two patients only. Infection was disseminated on different parts of the body in nine patients. In 39% of patients, infection was diagnosed in children younger than 9 years. Face and scalp infection was more often observed in children. The incidence was the highest during July and October. Contacts with soil and domestic animals were often reported. Data on the prevalence and clinical characteristics of M. gypseum complex infection in other countries are reviewed.

Background: Inherited epidermolysis bullosa (EB) comprises a highly heterogeneous group of rare diseases characterized by fragility and blistering of skin and mucous membranes. Clinical features combined with immunofluorescence antigen mapping and/or electron microscopy examination of a skin biopsy allow to define the EB type and subtype. Molecular diagnosis is nowadays feasible in all EB subtypes and required for prenatal diagnosis. The extent of skin and mucosal lesions varies greatly depending on EB subtype and patient age. In the more severe EB subtypes lifelong generalized blistering, chronic ulcerations and scarring sequelae lead to multiorgan involvement, major morbidity and life-threatening complications. In the absence of a cure, patient management remains based on preventive measures, together with symptomatic treatment of cutaneous and extracutaneous manifestations and complications. The rarity and complexity of EB challenge its appropriate care. Thus, the aim of the present study has been to generate multicentre, multidisciplinary recommendations on global skin care addressed to physicians, nurses and other health professionals dealing with EB, both in centres of expertise and primary care setting., Methods: Almost no controlled trials for EB treatment have been performed to date. For this reason, recommendations were prepared by a multidisciplinary team of experts from different European EB centres based on available literature and expert opinion. They have been subsequently revised by a panel of external experts, using an online-modified Delphi method to generate consensus., Results: Recommendations are reported according to the age of the patients. The major topics treated comprise the multidisciplinary approach to EB patients, global skin care including wound care, management of itching and pain, and early diagnosis of squamous cell carcinoma. Aspects of therapeutic patient education, care of disease burden and continuity of care are also developed., Conclusion: The recommendations are expected to be useful for daily global care of EB patients, in particular in the community setting. An optimal management of patients is also a prerequisite to allow them to benefit from the specific molecular and cell-based treatments currently under development.

Inverse psoriasis is a rare form of psoriasis characterized by the involvement of skin fold areas rather than the more common psoriatic involvement of the extensor surfaces of the extremities, trunk, and scalp. In addition, it requires a modified therapeutic approach because it is often less responsive to standard treatment regimens. Current treatment recommendations for inverse psoriasis mainly consist of topical agents, including corticosteroids, calcipotriol, and immunomodulating agents, whereas systemic medications remain insufficiently studied. Although adalimumab, a TNF-α inhibitor, has been approved for the treatment of moderate to severe plaque psoriasis, some reports indicate that TNF-α inhibitors may sometimes trigger psoriatic lesions, including inverse psoriasis. However, we present a case of inverse psoriasis and psoriatic arthritis unresponsive to standard treatment that was successfully treated with adalimumab.

Figurate erythema can appear in a wide spectrum of dermatological diseases. Rarely, it can present as an atypical manifestation of bullous pemphigoid. Among eosinophilic dermatoses, figurate erythema may appear in Wells syndrome, which has been occasionally reported in association with Toxocara infection. We present the case of an older female patient diagnosed with bullous pemphigoid, who presented with an unusual combination of blisters and figurate erythema outside the area of blister formation. In addition, high blood eosinophilia associated with lymph node and bone marrow eosinophilia was diagnosed and was causally related to Toxocara canis infection. The patient was treated with dapsone for bullous pemphigoid and with albendazole for toxocariosis, with complete regression of all skin lesions and blood eosinophilia. This paper discusses the possible etiopathogenesis of figurate erythema in our patient and summarizes previous clinical and histological findings in bullous pemphigoid and eosinophilic dermatoses presenting with figurate erythema lesions.