Dermatology online journal最新文献

A 35-year-old woman was initially diagnosed with classic dermatomyositis (DM) at age 21. We cared for her during 11 of those 14 years. Traditional immunosuppressive treatments did not control her DM skin disease activity. She was subsequently observed to have a juvenile-onset DM phenotype. For the past 11 years, her skin and muscle disease activity had been only partially controlled with monthly high-dose intravenous immunoglobulin (IVIG) therapy. During those 11 years of IVIG therapy, her skin disease activity continued to flare intermittently, and she developed cutaneous calcinosis. Both refractory skin disease activity and cutaneous calcinosis are known poor prognostic indicators for DM patients. Preliminary evidence suggests that the oral targeted synthetic Janus kinase inhibitor, tofacitinib, can provide more complete suppression of DM disease activity and possibly reverse cutaneous calcinosis. Furthermore, better suppression of DM disease activity could lower the patient's risk of future DM comorbidities, such as premature atherosclerotic cardiovascular disease. We propose transitioning the patient from monthly high-dose IVIG to daily oral tofacitinib therapy. Another potential benefit for both the patient and society could be substantial healthcare cost savings.

Corticosteroids are effective and relatively inexpensive medications used worldwide for multiple inflammatory diseases. Their use is marred by several well-established side effects, including hyperglycemia, weight gain, osteoporosis, monomorphic corticosteroid-induced acne, gastrointestinal disturbances, cataract, and glaucoma. We report a 29-year-old man with pemphigus vulgaris who achieved disease control with high-dose oral prednisolone and mycophenolate mofetil. While on treatment, he developed blurring of vision from 1 eye and was found to have central serous chorioretinopathy (CSCR). The condition was attributed to high-dose corticosteroid use by the ophthalmologist, who advised discontinuation of the offending drug as soon as possible. A rapid taper of corticosteroids was initiated to preserve vision while maintaining disease control. To our knowledge, CSCR has not been previously described in a patient with pemphigus vulgaris or other cutaneous immunobullous disorders in the English medical literature. This case aims to raise awareness of this rare ocular complication, which, if left untreated, can result in permanent vision loss.

Trigeminal trophic syndrome (TTS) is a rare consequence of central or peripheral trigeminal nerve injury, characterized by the classic triad of unilateral anesthesia, paresthesia, and ulceration. Most cases are preceded by iatrogenic injury to the trigeminal nerve. Other causes include stroke, head trauma, intracranial tumors, herpes virus infection, and degenerative central nervous system illnesses. Some cases are idiopathic. Limited information exists regarding pathogenesis, although neurotrophic factors and altered sympathetic activity are implicated. Self-mutilation is widely considered pivotal. We report a patient with TTS who presented with numbness, paresthesia, and a unilateral ulcer of the right forehead following herpes zoster infection.

Porokeratosis palmaris et plantaris disseminata (PPPD) is an exceptionally rare variant of porokeratosis characterized by abnormal clonal proliferation of keratinocytes along with distinctive histopathologic features-most notably, the cornoid lamella. It exhibits highly polymorphic clinical manifestations that pose significant diagnostic challenges. A 21-year-old man with PPPD presented with asymptomatic keratotic papules on the palms, soles, trunk, and limbs. Dermoscopy proved invaluable in guiding the diagnostic process, revealing annular structures interrupting the dermatoglyphics, with a brownish central clod and a whitish peripheral rim corresponding histologically to the cornoid lamella. The patient demonstrated significant improvement with oral acitretin and topical urea-based creams. This case illustrates the clinical nuances of PPPD, expands current dermoscopic insights into this rare entity, and underscores the critical role of dermoscopy in differentiating PPPD from other conditions in the differential diagnosis and in guiding biopsy procedures.

Cystic cutaneous lymphatic malformations are developmental anomalies of the lymphatic system characterized by lymphatic vessel dilatation. Microcystic forms, also known as circumscribed lymphangiomas, present as clusters of translucent or hemorrhagic vesicles, papules, or infiltrated plaques, often appearing in early childhood and primarily affecting the proximal extremities. Although generally asymptomatic, they may cause inflammatory, infectious, or hemorrhagic episodes. We report a 39-year-old woman with a 2-year history of vesicular lesions on the medial surface of both labia majora associated with intermittent pruritus and fluid leakage. Clinical examination revealed bilateral painless swellings, each approximately 6 cm, with translucent and hemorrhagic vesicles. Histology confirmed bilateral subcutaneous microcystic lymphangiomas. Given their benign nature, no treatment was recommended aside from surveillance. Microcystic lymphangiomas have an unpredictable course, with some remaining stable and others developing complications requiring treatment. Management depends on lesion location, symptoms, and esthetic impact, with options including sclerotherapy, CO₂ laser, or surgical excision, if necessary. This case highlights the importance of accurate diagnosis and individualized treatment of microcystic lymphangiomas.

Granulomatosis with polyangiitis (GPA) is an uncommon form of antineutrophil cytoplasmic antibodies-associated necrotizing vasculitis characterized by symptoms affecting the ears, nose, and throat, along with respiratory issues and kidney involvement. Although GPA is extremely rare among children, delayed diagnosis can have fatal consequences. We report a unique instance of GPA in a 15-year-old girl exhibiting solitary pyoderma gangrenosum-like ulcers on the face. After an extensive evaluation, the patient was diagnosed with GPA and demonstrated an excellent response to dual therapy combining rituximab and corticosteroids. Although exceptionally uncommon, clinicians must consider GPA as a possible condition in the differential diagnosis of individuals presenting with pyoderma gangrenosum-like ulcers.