Multiple acquired smooth muscle hamartomas with thick-walled blood vessels on scalp.

Smooth muscle hamartoma (SMH) is a benign hamartomatous condition that consists of a dermal proliferation of smooth muscle cells. We hereby report a case of multiple, acquired SMHs on scalp. A 25-year-old male had a 6-year history of multiple, asymptomatic, hyperpigmented plaques on the scalp which were progressively increasing in number and size. Histopathological examination revealed large thick-walled blood vessels, in addition to the haphazardly arranged discrete smooth muscle bundles in the reticular dermis. This case of SMH is unusual in being acquired, having multiple lesions, involving scalp, and having thick-walled blood vessels apart from arrector pili muscle bundles. It emphasizes the wide spectrum of clinical and histopathological variations that may be associated with acquired SMH.

Lipoid proteinosis: A case with distinct histopathological and radiological findings.

Lipoid proteinosis is a rare inherited genodermatosis characterized by hyaline deposits in various tissues. Clinically, it manifests with cutaneous as well as extracutaneous features. Periodic acid-Schiff (PAS)-reactive hyaline deposits in the upper dermis, with localization around blood vessels and eccrine sweat glands, in particular, is the histopathological hallmark finding. On brain imaging, bilateral symmetrical temporal lobe calcifications are considered to be pathognomonic of this disorder. We report a case of lipoid proteinosis in which hyaline deposits were present in the papillary and reticular dermis, without being seen at the periphery of eccrine sweat glands, along with dystrophic calcification. Magnetic resonance imaging (MRI) of brain revealed hydrocephalus, subependymal heterotropia and absent splenium of corpus callosum with no evidence of temporal lobe calcification. Thus, our case highlights the inherent diverse nature of lipoid proteinosis.

Cutaneous plasmacytosis with mast cell infiltration.

Cutaneous plasmacytosis is a rare disorder of uncertain etiology, described mainly in patients of Japanese descent. Clinically, it is characterized by multiple pigmented papules and plaques distributed primarily on the trunk. Histopathologically, it is marked by a dense dermal plasma cell infiltrate. Here, we describe a case of cutaneous plasmacytosis in a 55-year-old Indian male who presented with hyperpigmented plaques on the body. Histopathological examination revealed dense superficial and deep perivascular and periappendageal infiltrate composed mainly of plasma cells, lymphoid follicles with reactive germinal centres, perineural distribution of plasma cells, mast cell infiltration and increased dermal small blood vessels. Immunohistochemical analysis confirmed the polyclonal nature of the plasma cells. Laboratory investigations were within normal limits, except for the presence of polyclonal hypergammaglobulinemia without any M band. There was no evidence of autoimmune disease or any infection. There was no systemic involvement in this patient. The patient was diagnosed as cutaneous plasmacytosis and advised long-term follow-up. Peculiar histopathological finding in this case of cutaneous plasmacytosis was the presence of abundant mast cells in the dermis.

Localized unilateral basaloid follicular hamartoma along Blaschko's lines on face.

Basaloid follicular hamartoma (BFH) is a rare hamartoma of hair follicle. Clinical presentations may vary but are united by the same histopathological features in the form of folliculocentric basaloid or squamoid cell proliferation in the superficial dermis, which represents malformed and distorted hair follicles. It is important to recognize this entity as its simulant is basal cell carcinoma, a low-grade malignancy. Here, we report a case of localized unilateral BFH in a Blaschkoid distribution on the face of a 14-year-old female.