Ashim Kumar Mondal

Angiokeratoma circumscriptum neviforme: An entity, few and far between

Angiokeratomas are a group of vascular ectasias that involve the papillary dermis and may produce papillomatosis, acanthosis and hyperkeratosis of the epidermis. Angiokeratoma circumscriptum is the least common variant among many types. Angiokeratoma circumscriptum neviforme is a still rarer variety of angiokeratoma, which is classically seen at birth. We report here a case of congenital, linear, unilateral, verrucous plaques on the leg of a young girl, diagnosed as angiokeratoma circumscriptum neviforme (ACN).

Granulosis rubra nasi: a rare condition treated successfully with topical tacrolimus

A 20 years-old girl presented with multiple asymptomatic reddish vesicles on face for four years. It used to get worse in summer and was associated with localized hyperhidrosis. The lesions were notable for disappearance on diascopy. Histopathology from the vesicle showed mononuclear cell infiltration in the upper dermis, especially around eccrine sweat apparatus, along with dilatation of superficial capillaries and lymphatics. Based on clinical presentation and histopathology, diagnosis of Granulosis rubra nasi (GRN) was made. GRN usually resolves at puberty; however, rarely it may persist in adulthood. We here report a case of GRN having lesions persisting in adulthood. Moreover, she showed excellent response to topical tacrolimus, a finding not observed in literature.

A lady presenting with segmental anhidrosis and heat intolerance.

Sir, Anhidrosis can be the presenting symptom in a plethora of disorders, more common ones being leprosy, diabetic neuropathy, thyroid dysfunction, Sjögren syndrome, ectodermal dysplasia, autoimmune diseases, following anticholinergic medications etc., Herein we present the case of a middle-aged female who reported that the right side of her face was warmer than the left along with increased tendency for sweating.

A baby with photosensitivity and red teeth

Indian Dermatology Online Journal July-September 2013 Volume 4 Issue 3 CEP from hepatoerythropoietic porphyria (HEP), familial form of porphyria cutanea tarda (PCT), harderoporphyria and erythropoietic protoporphyria (EPP), which can present in a similar manner, is based on estimation of different porphyrins in the RBC, urine and stool. Earlier, erythrodontia was considered pathognomic of CEP.[2] Recently, it has been described in HEP, EPP and familial PCT as well.[3] Erythrodontia as an isolated finding too has been described in babies born to CEP mothers. Transplacentally acquired transient porphyria was suggested as the underlying mechanism.[4]

A boy with claw fingers.

An 8 years-old boy, presenting with clawing of left little and ring fingers for 2 years was referred to Department of Dermatology for evaluation of Hansen’s disease. There was no history of trauma or loss of sensation in affected limb or elsewhere, but had history of skin tightening of left limb and few patchy areas on trunk 2 years back, which resolved within a year. The clawing of fingers followed this skin tightening. Examination revealed no hypopigmented hypoaesthetic lesions anywhere on the body and no peripheral sensory loss was appreciable. The left hand was atrophic on medial side and was notable for linear indurated lesion extending from medial arm to palm and medial two fingers. Overlying skin was hyperpigmented and could not be pinched off. Similar indurated plaques were found on trunk and medial thighs too (Fig.1). All peripheral nerve trunks appeared to be normal in thickness. A clinical, diagnosis of Morphea (Linear morpheahand lesion and classical plaque typetrunk lesions) was made. Histopathology was consistent with the diagnosis.