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2003:69:7;15-16

Acquired ichthyosis with hoffmans syndrome

BD Sathyanarayana, B Leelavathi
 Department of Skin and S.T.D Govt. Medical College, Bangalore, India

Correspondence Address:
B D Sathyanarayana
Dept. of Skin & S.T.D, K.R. Hospital, Mysoer, Karnataka-5700 21
India
How to cite this article:
Sathyanarayana B D, Leelavathi B. Acquired ichthyosis with hoffmans syndrome. Indian J Dermatol Venereol Leprol 2003;69:15-16
Copyright: (C)2003 Indian Journal of Dermatology, Venereology, and Leprology

Abstract

A middle aged man presented with features of acquired ichthyosis with Hoffmans syndrome. Laboratory tests support hypothyodism. Myoedema and hypertrophy of muscles were present. Patient was previously treated for Pellagra.
Keywords: Acquired ichthyosis, Hypothyrodisn, Hoffman's Syndrome

Introduction

Hypothyrotaism may result from several causes of which autoimmune and iatrogenic are the most common. Muscular hypertrophic changes seen in hypothyroid patient is called Hoffman′s Syndrome.[1]

Malignancy like Hodgkins; drugs like cholesterol lowering and antipsychotic; autoimmune and nutritional deficiency disorders; infectious diseases like H.I.V, and endocrine disorders like hypothyroidism are the common disorders that can cause acquired ichthyosis.[2]

Case Report

A 50-year-old mule farmer, presented with complaints of generalised dryness of skin with mild itching, he had taken treatment for pellagra few years back.

The scales were present prominently over the extensor and exposed parts sparing the flexures. Palms and soles were thick and hyperkeratotic with spares body hair. Tongue was thick and enlarged. His speech was slow and slurred. He had sallow complexion.

Patient′s movements were sluggish. His calf muscles were bulky and tender. Deep tendon reflexes were normal but the ankle reflex had prolonged relaxation phase (hung-up sign). On percussion ridging of the muscles was present (myoedema).

Patient experienced a decline in memory power with gradual weakness and stiffness of limbs since few years.

Positive laboratory findings included - ESR : 80 mm/hr, serumtriglyceride : 377 mg/d1, increased TSH and decrease in T3 and T4 levels, C.P.K : 2966/liter, Electromyogram showed decreased conduction. Diagnosis of acquired ichthyosis with Hoffman′s syndrome was made.

Discussion

Muscle cramps, stiffness and weakness frequently seen in hypothyroidism are due to a primary myopathy.[3] Muscle hypertrophy is a rare occurrence in hypothyroidism. It is more commonly seen in males than females and is referred as Hoffman′s syndrome in adult and Kocher-Debre-Semelaigne syndrome in children.[4] Mounding phenomenon (myoedema) which may occur when muscle is directly percussed and slow relaxation of reflexes are associated features in Hoffman′s syndrome. Thyroid replacement may cause improvement in hypothyroid myopathy but such improvement may be incomplete in severe case.[5]

In the absence of typical exposure dermatitis in pellagra, one should always rule out the possibility of hypothyroidism.

References
1.
Moxley RT. Metabolic and endocrine myopathies in disorders of voluntary muscles, editors. Walton J Karpati G, Jones D.H, 6th ed, Churchill Livingstone. 1994; p691.
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2.
Digiovanna JJ : Ichthyosiform dermotoses, in : Dermatology in General Medicine, Editors, Freedberg IM, Eisen AZ, Wolffk etal. 5th ed, Mc Graw-Hill, Vol 1, 1999; 594-595.
[Google Scholar]
3.
Kendall-Taylor P, Turnbull DM, Endocrine myopathy, Br Med J 1983; 287:705-708.
[Google Scholar]
4.
Riggs JE, hypothyroidism, in Neurology in Clinical Practice, Vol II, editors, Bradley W G, Daroff RB, Fenichel G M et al, Butterworth-Heinemann (USA), 1991; B848-849.
[Google Scholar]
5.
Bolton CF, Myopathi, in; Clinical Electromyography. Editors, Brown WF, Bolton CF, 2nd ed, 1993.
[Google Scholar]
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