Dowling–Degos disease (DDD) and reticulate acropigmentation of Kitamura (RAK) are rare genodermatoses inherited as an autosomal dominant trait with variable penetrance. They are considered to be part of a spectrum of reticulate pigmentary dermatoses, characterized by the presence of hyperpigmented macules coalescing in a reticular fashion. The authors describe a 28-year-old male patient having hyperpigmented macules on the axillae, neck and face, reticulate acropigmentation of dorsum of the hands, forearms and feet, palmar pitting, and comedo-like lesions over back. The patient showed the unique clinical as well as histopathological overlap of both the rare diseases (DDD and RAK), substantiating the hypothesis that they represent two different features of a single entity with variable phenotypic expression.
Introduction: Cutaneous appendageal tumors are a large diverse group of tumors that are commonly classified according to their state of appendageal differentiation-eccrine, apocrine, follicular and sebaceous. Most appendageal tumors are relatively uncommonly encountered in routine clinical practice. Though some of the appendageal tumors (e.g. syringoma, nevus sebaceous) can be diagnosed clinically with ease but most of them have non-specific morphological appearance and their diagnosis is mainly based on histopathological characteristics. Material and methods: It was a cross sectional descriptive study conducted in the department of dermatology, Chirayu medical college and hospital, Bhopal. A total of 90 patients attending department of dermatology were screened out for appendageal tumors and those (60 patients/66.6%) who were confirmed by histopathological examination of lesional biopsy were included in the study. Results: On the basis of differentiation of tumors, sweat gland tumors were the most common appendageal tumors in our study (79.8%) followed by follicular tumors (11.6%), sebaceous were 8.3%. On individual tumor basis, syringoma constituted maximum numbers of cases (56.6%) followed by multiple trichoepitheliomas and hidrocystoma (both 11.6%). Face was the commonest site of predilection 40(66.6%) followed by scalp 12(20%). Conclusion: Appendageal tumors are relatively uncommon and constitute very small proportion of dermatological disorders. Though clinical appearance might help in diagnosing common appendageal tumors but histological examination remains the gold standard method in diagnosing clinically suspicious lesions.
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