Shriya Dave

Disseminated and Disfiguring Molluscum Contagiosum in a Child

Abstract: We report widely disseminated and disfiguring lesions of molluscum contagiosum occurring in a 4‐year‐old girl secondary to a primary immunodeficiency disorder. The child, born of consanguineous parents, had multiple, asymptomatic, raised, skin‐colored and whitish lesions since 2 years of age. On physical examination she had growth retardation features with grade II protein energy malnutrition. Cutaneous examination revealed numerous, widespread, skin‐colored to translucent, firm, umbilicated papules of varying sizes (pinpoint to larger than 10 mm). They were distributed bilaterally over all four limbs, including the dorsum of the hands and feet, trunk, face (including lips), eyelids, auriculae, and perineal and gluteal areas. In places, they were linearly disposed and had coalesced to form plaques. A biopsy specimen from a representative skin lesion (arm) demonstrated lobulated epidermal growth consisting of keratinocytes with large intracytoplasmic eosinophilic inclusion bodies and a central crater. These findings were consistent with the diagnosis of molluscum contagiosum. Topical therapies with phenol and trichloroacetic acid were ineffective The child succumbed to a fulminant systemic infection at home, 2 months after discharge from the hospital. The extent and distribution of molluscum contagiosum in our patient was remarkably vivid and has been hitherto reported only rarely.

Griscelli Syndrome : A Rare Immunodeficiency Disorder with Partial Albinism

To the Editor: Griscelli syndrome is an immunodeficiency first described in 1978 by Dr. Claude Griscelli, who worked at “Hopital Necker-Enfants Malades” in Paris, France (1). It is characterized by partial oculocutaneous albinism, variable cellular and humoral immunodeficiencies and the occurrence of “accelerated phases” consisting of haemophagocytosis, pancytopenia, elevation of serum triglyceride levels, hypofibrinogenemia with associated bleeding diathesis, and hypoproteinemia (2). The cutaneous features are dominated by presence of silver-gray hair including silver colored hair over the eyebrows and eyelashes which show pigment clumps on light microscopy. This syndrome is extremely rare; since its original descriptoin, only about forty cases have been described in the literature (3). Most cases reported belong to the Turkish and Mediterranean populations; and to the best of our knowledge, there is no previous report of Griscelli syndrome occuring in the Indian population. A twelve-year-old girl was referred to us by the department of pediatrics for evaluation of altered hair and skin pigmentation. The child had presented to them with a history of fever with chills, cough, and weight loss. On systemic examination, she was found to have marked pallor, icterus, lymphadenopathy, and hepatosplenomegaly. Dermatological examination revealed silvery gray to golden hue of hair over the scalp, eyebrows, and eyelashes (Fig. 1). A silvery sheen was also present over most of the body hair. There was brownish-black pigmentation of the skin over the sun-exposed sites, especially on the face and extensor aspects of the upper limbs. The skin in the covered areas of the body was comparatively light colored. A detailed history revealed that the patient had had silvery gray to golden hair since birth and had developed the hyperpigmentation gradually over the years. A few purpuric lesions were present over the lower limbs. Ophthalmological examination revealed a visual acuity of 6/9 (right eye) and 6/18 (left eye). Examination of the fundus revealed mild vascular sheathing with features of papilledema. There was a past history of a severe illness with fever and seizures at the age of six years for which the patient The Journal of Dermatology Vol. 31: 247–250, 2004