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LETTER TO THE EDITOR
Year : 2014  |  Volume : 5  |  Issue : 4  |  Page : 522-523  

Mal de Meleda: A report of two siblings in one family


Department of Skin and VD, JNMC, Belgaum, Karnataka, India

Date of Web Publication10-Oct-2014

Correspondence Address:
Vijaya V Sajjan
Department of Skin and VD, JNMC, Belgaum, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.142538

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How to cite this article:
Sajjan VV, Taj F, Manjunathswamy B S. Mal de Meleda: A report of two siblings in one family . Indian Dermatol Online J 2014;5:522-3

How to cite this URL:
Sajjan VV, Taj F, Manjunathswamy B S. Mal de Meleda: A report of two siblings in one family . Indian Dermatol Online J [serial online] 2014 [cited 2019 Dec 6];5:522-3. Available from: http://www.idoj.in/text.asp?2014/5/4/522/142538

Sir,

Two siblings female and male of age 12 and 10 years born out of a second degree consanguineous marriage presented with a small patch of asymptomatic peeling of the skin over the palms since birth. The patch started increasing at the age of nine months to involve both palms and soles and spread to the dorsal aspect of hands, feet, and extensor knees and elbows. Developmental milestones were normal and performance in school was above average. There was no history of hearing impairment, eye problem, or dental caries.

On examination, both the siblings showed diffuse palmoplantar keratoderma (PPK) extending proximally up to the wrist and dorsum of hands and feet with well-defined margins, in a transgrediens pattern [Figure 1], [Figure 2], [Figure 3], [Figure 4]. The skin over the palms and soles was thick and erythematous with loss of dermatoglyphics and fissures. The severity of skin lesions was more in the male sibling. Hyperhidrosis of palms and soles was also noted in the male. Well-defined erythematous plaques were present over the extensor aspect of the elbow and knee joint. The nails were normal. Histopathology of skin lesions showed hyperkeratosis, hypergranulosis and acanthosis and a perivascular mononuclear cell infiltrate in the upper dermis.
Figure 1: Transgradient palmoplantar keratoderma with sharp margins in the girl sibling

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Figure 2: Transgradient palmoplantar keratoderma with sharp margins in the girl sibling

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Figure 3: Transgradient palmoplantar keratoderma with sharp margins in the boy

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Figure 4: Transgradient palmoplantar keratoderma with sharp margins on both knees in the boy

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The PPKs are a heterogenous group of disorders, characterized by thickening of the skin of the palms and soles. They can be categorized as inherited and acquired, or subdivided based on clinical patterns. Mal de Meleda (MDM), one of the autosomal recessive forms of PPK, is clinically characterized by a well-demarcated erythema and hyperkeratosis of the palms and soles that usually occurs soon after birth. The hyperkeratosis spreads slowly to the dorsal aspects of the hands and feet, which is referred to as transgrediens. Many of the patients will experience bothersome pain due to fissures. Hyperhidrosis with maceration is feature of MDM and is often accompanied by malodour. In addition, patients may develop keratotic plaques over joints, nail abnormalities, brachydactyly, pseudoanihum and perioral erythema. [1] In 2001, mutations in the gene encoding secreted Ly-6/uPAR-related protein 1 (SLURP-1) located on the chromosome 8q24.3 were found to be the cause of MDM. It is currently identified that SLURP-1 is an epidermal secreted neuromodulator that influences both epidermal homeostasis and inhibition of tumor necrosis factor-alpha release by the macrophages during the wound healing process. Such roles would explain the hyperproliferative and inflammatory clinical characteristics of MDM. Both our cases showed the following compatible clinical features: "glove-and-socks" distribution of the keratoderma with a sharp demarcation that appeared after birth and progressively extended to the dorsa of the hands and feet and palmoplantar hyperhidrosis. Both siblings were treated with acitretin 10 mg and improvement was noted within one month.

MDM must be differentiated from other syndromes as shown in [Table 1].[5]
Table 1: Differential diagnosis of MDM

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   References Top

1.
Nath AK, Chaudhuri S, Thappa DM. Mal de meleda with lip involvement: A report of two cases. Indian J Dermatol 2012;57:390-3.  Back to cited text no. 1
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2.
Flückiger R, Itin PH. Keratosis extremitatum (Greither's disease): Clinical features, histology, ultrastructure. Dermatology 1993;187:309-11.  Back to cited text no. 2
    
3.
Shah J, Goel S. Papillon-Lefevre syndrome: Two case reports. Indian J Dent Res 2007;18:210-3.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.
Cavalcante LI, Holanda EM, Almeida TL, Accioly-Filho JW. Ceratodermia mutilante de Vohwinkel: Relato de três casos em uma família. An Bras Dermatol 2003;78:311-8.  Back to cited text no. 4
    
5.
Cesarini LV, Pegas JR, Reis VM, Müller H, Oliveira MA, Pires MC. Ceratodermia palmoplantar de Unna-Thost associada a pseudo-ainhum-Relato de um caso. An Bras Dermatol 2004;79:61-7.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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