ABSTRACT
A 34-year-old woman presented to our outpatient clinic with photosensitivity, photophobia, and facial pruritus (Figure 1). She had brown eyes and fair skin, hair, eyelashes, and eyebrows since birth. Her sister had similar skin and hair pigments. The patient had no systemic disease and was not taking any medication. Her parents were second-degree relatives. A dermatologic examination revealed small hyperkeratotic papules with an erythematous background, minimal desquamation, and some excoriation over the nose, zygomatic arch, and forehead consistent with actinic keratosis and solar damage. An ophthalmological examination demonstrated impaired visual acuity (60/100 in both eyes, reaching 80/100 in the left eye with best correction). Hypopigmentation at the albinotic retinal midperiphery (Figure 2) by fundoscopy was noted. She had no nystagmus or strabismus. The patient had no complaints or symptoms of the neurological, gastrointestinal, or respiratory system, and she had no recurrent skin or systemic infection.
Subject(s)
Hermanski-Pudlak Syndrome/diagnosis , Adult , Diagnosis, Differential , Female , Hermanski-Pudlak Syndrome/complications , Hermanski-Pudlak Syndrome/physiopathology , Humans , Keratosis, Actinic/complicationsABSTRACT
BACKGROUND: Acquired perforating dermatosis (APD) is a rare group of skin disorders characterized by transepidermal elimination of dermal tissue material. There are only a few studies of APD. OBJECTIVE: We aimed to review the clinicopathologic features of patients with APD and evaluate the outcomes of treatments. MATERIAL AND METHODS: The medical records of 25 patients who were clinically and histopathologically diagnosed as having APD between 2006 and 2013 were retrospectively reviewed. The histopathologic sections and the results of elastic, trichrome and periodic acid Shiff (PAS) staining were evaluated. RESULTS: Of the patients, 17 (68%) had ARPC, 7 (28%) had perforating folliculitis, 1 (4%) had Kyrle's disease. Male/female ratio was 1.3. The mean age was 51.8 ± 12.8 years. The disease duration ranged between 15 days and 96 months. Diabetes mellitus was the most common disease associated with APD (48%). The walls of vessels were PAS positive in the lesions of 6 of 13 diabetic patients. After various treatments, almost all lesions regressed in 15 (60%) patients. CONCLUSION: Acquired perforating dermatosis may present with various clinical features and is mostly associated with diabetes mellitus. Diabetes mellitus may be involved in the pathogenesis of APD via microangiopathy and other mechanisms.
