Translate this page into:
V K Jain
11/9J, Medical Enclave, Pt. BDS PGIMS, Rohtak-124001 (Haryana)
|How to cite this article:
Brahma D, Jain V K, Aggarwal K. Erythrokeratoderma variabilis. Indian J Dermatol Venereol Leprol 2003;69:5-6
AbstractA case of erythrokeratoderma variabilis (EKV) in a male patient, who presented with reddish-brown, well-defined, hyperkeratotic, bilaterally symmetrical plaques on the extensor aspects of upper and lower extremities accompanied by migratory, erythematous patches forming bizarre geometric pattern over the rest of the body which varied in number, size and shape, is being reported. Patient also had palmoplantar keratoderma. Family history was negative. Hisotpathology confirmed the diagnosis of EK.
Erythrokeratodermias (EK) are a heterogenous group of inherited cornification disorders. EK were classified as i) confluent and reticular EK of Gougerot and Carteaud, ii) variable EK of Mendes da Costa and iii) symmetric progressive EK. Erythrokeratoderma variabilis (EKV) is a rare, ichthyosiform disorder, first, described by Mendes da Costa in 1925. Clinically, the lesions, which are of two types, usually appear in the early months of life but may occasionally develop considerably later. There are dark, erythematous, well-demarcated plaques, symmetrically arrayed, relatively fixed in site, showing a predilication for the extensor surfaces. There are also transient lesions consisting of distinctive polycyclic or comma-shaped erythematous macules, occurring at any site, which last days or weeks and may either fade in situ or migrate slowly over the surface followed by fine scale. Additionally, hypertrichosis of vellus hair and mild keratoderma of palms and soles may be present. Histopathological studies show that the lower most portion of the stratum corneum is compact while the majority of the stratum corneum exhibits a loose basket-weave pattern. Other features are papillomatosis, hyperplasia of ridges and a perivascular lymphohistiocytic infiltrate in the papillary dermis. However, the ultrastructural finding of subnormal numbers of epidermal keratinosomes within hyperkeratotic plaques is characteristic of this disorder.9
A 30 year old male had reddish-brown plaques since the last 25 years. The plaques were thickened, brown, pigmented and sharply delineated. Also noted at that time were asymptomatic islands of configurate erythema on the trunk and extremities which varied in size, position, shape and number. On examination, well-defined, reddish-brown, hyperkeratotic, bilaterally symmetrical plaques were present over the elbows, knees and ankles. These lesions showed accentuation of skin creases and have been fixed in their site since onset. This was accompanied by transient, erythematous lesions consisting of discrete, amoeboid margined, finely scaly erythematosus patches of variable size, with bizarre geographical configuration, distributed irregularly over the upper and lower extremities, front and back of trunk with relative sparing of face. These lesion were mildly itchy, migratory in nature, lasting days or weeks and subsequently either fading with yellowish-brown scaling or involuting completely and appearing at new sites, thereafter. There was no history of exacerbation of these lesions following exposure to cold, heat, wind or emotional upset. The patient′s general health was good and he was not receiving any medication. The palms and soles exhibited a mild, diffuse keratoderma. Family history did not reveal any evidence of this disorder. The nails, teeth and mucous membranes appeared normal. The remainder of the physical examination revealed no abnormalities. Complete and differential blood cell counts, liver function tests and urinary analysis gave results within normal limits.
EKV is a very rare type of EK, determined by a autosomal dominant gene of variable expressivity, characterized by reddish-brown plaques, which are hyperkeratotic and fixed in location, particularly, over the knees, elbows and ankles and figurate patches of erythema which vary in size, shape and location and that move around. In our patient, family history was negative, onset was around the age of 5 years and in addition to the typical plaques and migratory, circinate patches; mild diffuse palmoplantar keratoderma was present.
Nazzaro V, Bardon CB. Progressive symmetric erythrokeratoderma. Arch Dermatol 1986; 122:434-440.[Google Scholar]
Gertler W. Systematische Dermatologie und Grenzgebiete Thieme, Leipzig 1970; 1:181.[Google Scholar]
Mendes da Costa S. Erythro-et keratodermia variabilis in a mother and daughter. Acta Derm Venereol 1925; 6:255-258.[Google Scholar]
Hacham - Zadeh S, Even - Paz Z. Erythrokeratoderma variabilis in a Jewish Kuridish family. Clin Genet 1978; 13:404-408.[Google Scholar]
Belaich S, Homareau S, Blanchet - Bardon C et al. Erythrokeratoderma variable (Mendes da Costa). Etude ultrastructurale. Ann Dermatol Venereol 1988; 115:1121-1123.[Google Scholar]
Griffiths WAD, Judge MR, Leigh IM. Disorders of Keratinization, In: Textbook of Dermatology. Edited by Champion RH, Burton JL, Burns DA et al, Blackwell Science Ltd., London, 1998; 1533-1534.[Google Scholar]
Rappaport LP, Goldes JA, Goltz RW. Erythrokeratoderma variabilis treated with Isotretinoin. Arch Dermatol 1986; 122: 441-445.[Google Scholar]
Gerwirtzman GB, Winkler NW, Dobson RL. Erythrokeratoderma variabilis. A Family Study. Arch Dermatol 1978; 114: 259-161.[Google Scholar]
Vandersteen PR, Muller SA. Erythrokeratoderma variabilis: An enzyme histochemical and ultrastructural study. Arch Dermatol 1971; 103: 362-370.[Google Scholar]