No abstract

Epidermolytic ichthyosis (EI) is a rare genodermatosis disorder. We report a 39-year-old woman with EI, who presented with generalized erythroderma since birth, followed by generalized hyperkeratosis later in life. The physical examination revealed generalized hyperkeratosis without blistering or erosion. The histopathological studies revealed hyperkeratosis with parakeratosis and psoriasiform hyperplasia, without significant epidermolysis. The Sanger sequencing revealed a missense mutation—c.467G>A (p.Arg156His)—in the KRT10 gene, confirming the diagnosis of EI. The genotype-phenotype correlations in EI patients are multifactorial. Thus, molecular analysis can confirm the diagnosis in cases of an unclear medical history or histological inconclusiveness.

We present a case of febrile ulceronecrotic Mucha-Habermann disease who presented with widespread erythematous crusted papules, which rapidly progressed to ulceronecrotic lesions accompanied by fever. The serologies showed high titers of cytomegalovirus IgG (>1:3,200) and IgM (1:800). The histopathological study showed epidermal necrosis. The treatment was begun with systemic steroids and roxithromycin to which the patient did not respond. After switching the treatment to methotrexate, the patient was further complicated by getting hepatitis. Low dose cyclosporine resolved the situation within 2 weeks. In patients for whom methotrexate is contra-indicated or ineffective, cyclosporine can suppress T-lymphocyte hyperresponsiveness and resolve this disease.